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Standardized protocols have been shown to improve outcomes in several pediatric surgical conditions. We implemented a multi-disciplinary gastroschisis practice bundle at our institution in 2013. We sought to evaluate its impact on closure type and early clinical outcomes.We performed a retrospective review of uncomplicated gastroschisis patients treated at our institution between 2008-2019. Patients were divided into two groups: pre- and post-protocol implementation. Multivariate logistic regression was used to compare closure location, method, and success.Neonates (pre-implementation n = 53, post-implementation n = 43) were similar across baseline variables. Successful immediate closure rates were comparable (75.5% vs. 72.1%, p = 0.71). The proportion of bedside closures increased significantly after protocol implementation (35.3% vs. 95.4%, p 0.01), as did the proportion of sutureless closures (32.5% vs. 71.0%, p 0.01). Median postoperative mechanical ventilation decreased significantly (4 days IQR [3, 5] vs. 2 days IQR [1, 3], p 0.01). Postoperative complications and duration of parenteral nutrition were equivalent. After controlling for potential confounding, infants in the post-implementation group had a 44.0 times higher odds of undergoing bedside closure (95% CI: 9.0, 215.2, p 0.01) and a 7.7 times higher odds of undergoing sutureless closure (95% CI: 2.3, 25.1, p 0.01).Implementing a standardized gastroschisis protocol significantly increased the proportion of immediate bedside sutureless closures and decreased the duration of mechanical ventilation, without increasing postoperative complications. Level of Evidence III Type of Study Retrospective comparative study.

Background Wide practice variation exists in the management of gastroschisis. Routine endotracheal intubation for bedside closure may lead to longer duration of mechanical ventilation. Methods The Canadian Association of Pediatric Surgery Network gastroschisis dataset was queried for all patients undergoing attempted bedside reduction and closure. Patients with evidence of intestinal necrosis or perforation were excluded. A propensity score analysis was used to compare the rate of successful primary repair and post-operative outcomes between intubated and non-intubated patients. Results In propensity score matched analysis, the successful primary repair rate did not reach statistical significance between patients who were intubated for attempted bedside closure and those who were not intubated (Odds Ratio: 2.18, 95% Confidence Interval: 0.79, 6.03). Intubated patients experienced 3.02 more ventilator days than patients who were not intubated at the time of initial attempted closure. Other post-operative parameters were similar between both groups. Conclusions It is reasonable to attempt primary bedside gastroschisis closure without intubation in otherwise healthy infants.

Resume Nous rapportons le cas d’une volumineuse tumeur renale gauche avec remaniements necrotiques et hemorragiques chez un enfant de 7 mois, suggerant cliniquement et radiologiquement un nephroblastome. Il s’agissait d’une tumeur nodulaire de 8 cm, qui occupait les deux tiers du rein et presentait des plages necrotico-hemorragiques. Il n’etait constate ni rupture capsulaire, ni infiltration du sinus renal. Le parenchyme renal adjacent apparaissait macroscopiquement normal. A l’examen histologique, la tumeur presentait une architecture uniquement tubulopapillaire avec de nombreux psammomes. Le diagnostic differentiel principal retenu etait celui d’un nephroblastome epithelial pur. Toutefois, certaines caracteristiques histologiques et immuno-histochimiques ont permis d’infirmer cette hypothese et de retenir le diagnostic d’adenome metanephrique. L’adenome metanephrique est une tumeur renale benigne exceptionnelle chez l’enfant. Le pathologiste se doit d’y penser car le seul traitement necessaire est la chirurgie.

Purpose Effective antenatal counseling in congenital diaphragmatic hernia (CDH) relies on proper measurement of prognostic indices. This quality initiative audited the accuracy of prenatal imaging with postnatal outcomes at two tertiary pediatric referral centers. Methods Prenatal lung–head ratio (LHR) and total fetal lung volume (TFLV) for CDH patients treated between 2006 and 2017 were retrieved. Study inclusion required at least one LHR or TFLV measurement between 24 and 32 weeks gestational age. Postnatal outcomes [mortality, extracorporeal life support (ECLS) need, patch repair, persistent pulmonary hypertension, oxygen requirement at 28 days] were abstracted from the Canadian Pediatric Surgery Network (CAPSNet) database and local chart review. Univariate and descriptive analyses were conducted. Results Eighty-two of 121 eligible CDH patients (68%) were included. Overall mortality, ECLS rates, and patch repair were 33%, 12.5%, and 45%, respectively. Lower LHR values correlated with increased rates of each outcome and persisted despite multiple measurements. Values obtained were higher than those in published schemata. LHR values > 45% were most associated with survival, avoidance of ECLS, and primary repair. TFLV values only correlated with mortality and patch repair. Conclusions This audit confirms that LHR and TFLV values predict CDH outcomes. However, absolute values obtained require careful interpretation and internal review. Level of evidence IV.

Background: Children with gastroschisis, especially when it is complicated by atresia or perforation, often suffer from severe long-term sequelae such as short-bowel syndrome and chronic dysmotility. These children are also susceptible to adhesive bowel obstruction later in life, which can alter their nutritional intake and may require repeated visits to the hospital. However, few long-term studies have attempted to determine the rate of occurrence of intestinal occlusion after gastroschisis. In this study we measured the incidence and management of adhesive bowel obstruction in children with gastroschisis at one academic pediatric hospital. Methods: A retrospective chart review was carried out on patients with gastroschisis born between January 2000 and December 2007. A total of 74 patients who underwent closure of their gastroschisis during this period were identified. Results: Twelve of these 74 (16%) patients were readmitted to the hospital with at least one episode of adhesive bowel obstruction. Three of the 12 patients had gastroschisis complicated by atresia. Eight patients underwent immediate primary closure of their defect on the first day of life. Silos were installed on the remaining four (36%) and were kept in place for an average of 6.25 (range: 4–9) days. Five of the 12 children resolved their obstruction with nasogastric tube decompression alone. The remaining seven (58%) required lysis of adhesions after failure of decompression. The seven children who underwent surgical lysis of adhesions did not have additional episodes of occlusion after laparotomy. Conclusion: In this study, 16% of the patients born with gastroschisis within a 7-year period presented with a adhesive obstruction. Occurrence of obstruction was unrelated to the method of closure or the severity of the defect. Although conservative management with nasogastric tube decompression may be effective initially, over half of patients with occlusion will need eventual surgery for lysis of adhesions. Keywords: bowel obstruction, gastroschisis, lysis of adhesions

Vibratory rollers transmit vibrations that may lead to pore pressure build up and soil failure in vibration susceptible soils. In a recent incident at Statland in mid Norway, a vibratory roller had compacted a shoreline embankment fill shortly before a submarine landslide was initiated. The landslide triggered a tsunami causing economic damages for several millions NOK. This incident shows the need for guidelines for controlling the effects of construction activity induced vibrations on slope stability. In a follow up study, the effect of vibratory roller compaction on soil degradation has been evaluated with a finite element model. The effect of vibrations varies with parameters such as soil stiffness, bedrock depth and geometry, and presence of thin soft layers. To develop a vibration measurement procedure, we suggest, based on a threshold shear strain of 0.025%, a 15 m wide and 5 m deep influence zone outside of which the soil strength is not reduced. A tentative vibration limit is set to 10 mm/s near slopes with vibration susceptible materials. We also provide some recommendations on how to account for vibratory compaction in slope stability analysis.

This paper describes a detailed geotechnical site investigation carried out to study the static and dynamic properties of a post-glacial Goldthwait Sea sensitive clay deposit. The focus is on the evaluation of the small-strain shear modulus of the sensitive clay. The site is located on the North Shore of the St-Lawrence Gulf (Province of Quebec) and it is selected as it is the location of a major landslide triggered by rock blasting operations in 2009. The investigations involved both in-situ and laboratory tests (resonant column and bender element testing). The laboratory tests were carried out on high quality large diameter clay samples from three different depths. The objective of this paper is to compare the laboratory small-strain shear modulus obtained with resonant column and bender elements with the small-strain shear modulus obtained in the field using the seismic piezocone (SCPTu) and ambient vibrations.

Background Despite randomized controlled trials and meta-analyses, it remains unclear whether laparoscopic pyloromyotomy (LP) carries a higher risk of incomplete pyloromyotomy and mucosal perforation compared with open pyloromyotomy (OP). Methods Multicenter study of all pyloromyotomies (May 2007–December 2010) at nine high-volume institutions. The effect of laparoscopy on the procedure-related complications of incomplete pyloromyotomy and mucosal perforation was determined using binomial logistic regression adjusting for differences among centers. Results Data relating to 2830 pyloromyotomies (1802 [64%] LP) were analyzed. There were 24 cases of incomplete pyloromyotomy; 3 in the open group (0.29%) and 21 in the laparoscopic group (1.16%). There were 18 cases of mucosal perforation; 3 in the open group (0.29%) and 15 in the laparoscopic group (0.83%). The regression model demonstrated that LP was a marginally significant predictor of incomplete pyloromyotomy (adjusted difference 0.87% [95% CI 0.006–4.083]; P =0.046) but not of mucosal perforation (adjusted difference 0.56% [95% CI −0.096 to 3.365]; P =0.153). Trainees performed a similar proportion of each procedure (laparoscopic 82.6% vs. open 80.3%; P =0.2) and grade of primary operator did not affect the rate of either complication. Conclusions This is one of the largest series of pyloromyotomy ever reported. Although laparoscopy is associated with a statistically significant increase in the risk of incomplete pyloromyotomy, the effect size is small and of questionable clinical relevance. Both OP and LP are associated with low rates of mucosal perforation and incomplete pyloromyotomy in specialist centers, whether trainee or consultant surgeons perform the procedure.

We report the case of a large tumor in the left kidney with necrotic and hemorrhagic features in a 7-month-old child, which was clinically and radiologically suggestive of a nephroblastoma. The tumor was a nodular mass measuring 8cm in diameter occupying two thirds of the kidney and presenting areas of necrosis and hemorrhage. No capsular rupture or renal sinus infiltration were found. Adjacent renal parenchyma appeared mascroscopically normal. Histologically, the tumor showed a strictly tubulopapillary architectural pattern with numerous psammomas. The initial hypothesis was a purely epithelial nephroblastoma. However, this hypothesis was rejected due to some immunohistochemical and histological characteristics and the final diagnosis was a metanephric adenoma. Metanephric adenoma is an exceptionally rare benign renal tumor in children. However, pathologists need to keep it in mind because simple surgical excision is curative.

Perianal rhabdomyosarcoma is a rare type of tumor with a relatively poor prognosis. We present the case of a patient who presented with a cutaneous perianal hamartoma at the age of 6 weeks. 21 months latter a recurrent mass at the excision site proved to be an embryonal rhabdomyosarcoma involving the anal sphincter. A pathologic review of the two specimens confirmed their relatedness. This report highlights the need to maintain a high level of suspicion in cases of recurrence following excision of a benign lesion.

Purpose Megarectum complicating surgery for anorectal malformation (ARM) has implications for long-term continence. Factors influencing continence and defecation include intact rectal reservoir, innervation/proprioception of the anorectal muscle complex, functioning anorectal inhibitory reflex (AIR), and intact perception at the anal margin. We studied outcomes after surgery for ARM with emphasis on megarectum; particularly as to whether altered rectal proprioception from anatomic sacrococcygeal anomalies affect incidence. We also assessed whether an abnormal AIR could trigger passive rectal dilatation without mechanical obstruction. Methods Eighty six infants (53 male) with ARM over 20 years were included. Demographics, surgical history, pathology, defecation patterns, imaging, manometry, and morbidity were analyzed. Incidence of sacrococcygeal malformations in children with and without megarectum was compared using Fisher exact test. Manometry results were evaluated for integrity of AIR and correlated to megarectum occurrence. Results There were 23 high/intermediate and 63 low ARMs. Fourteen (16%) developed a megarectum: 6 of 23 in high and 8 of 63 in low anomalies ( P = .33). Twelve patients underwent megarectum resection at a median of 2.6 years (7 months to 10 years); 2 received bowel management protocols. Fifty-seven percent (8/14) of children with and 7% (5/72) without megarectum had sacrovertebral anomalies ( P = .0001). Patients with preoperative manometry (n = 5) demonstrated an intact AIR. Colonic manometry demonstrated hyperactive colons (n = 2). Constipation was the predominant preoperative symptom; 3 patients suffered from incontinence after resection. All the specimens showed normal innervation and thickened muscularis on pathology. Conclusions Sacral anomalies, which are more prevalent in children who developed megarectum, may result in abnormal rectal proprioception contributing to this pathology. Innervation anomalies may coexist, although preoperative manometries showed normal AIRs. Rectal dysmotility may lead to stool retention with subsequent dilatation, and patients who underwent colonic manometry had diffuse colonic hypermotility. Further physiologic and cellular studies are needed to elucidate the causes of this significant complication after surgical ARM repair in the absence of obstruction.

Primarily to study morbidity and mortality in jejuno-ileal atresias (JIA) and prognostic factors for outcome. Secondarily to look at the incidence of reintervention. Retrospective review of 63 patients diagnosed with JIA over a 30-year period (1975–2005). Sixty-three patients (34 male) of mean gestational age 36 weeks and mean birth weight 2,858 g with JIA were studied. There were 14 type I, 14 type II, 16 type IIIA, 9 type IIIB, and 10 type IV atresias. Thirty-three patients (52%) had associated anomalies. Fifty-one patients underwent resection and anastamosis, five patients Bishop–Koop procedure, five ileostomies, and one strictureplasty. Intestinal dilatation severe enough to warrant surgical intervention was seen in seven patients with the more severe variants of atresia. Five tapering procedures, one Bianchi operation and one STEP procedure were performed. Average hospital stay was 41 days (8–332 days). Fifty-six were alive at follow ups averaging 1.7 years (6 months to 11 years). Nine patients needed reoperations for adhesions before the first year of life. There were seven deaths. Most patients who died had associated anomalies (P = 0.017) or types IV/V atresias (P = 0.007). Mild atresias have an excellent prognosis and long-term survival. Severe atresias are associated with longer PN support and secondary procedures for intestinal failure. Associated anomalies adversely affect outcomes in JIA.

Neonatal cystic disorders of the lungs are a heterogeneous malformative group including giant lobar hyperinflation, congenital pulmonary airway malformations, intralobar pulmonary sequestration, and bronchogenic cyst. Here, we describe a giant cystic pulmonary malformation in a 5-year-old girl, morphologically characterized by a highly disorganized proliferation of numerous cartilage islands, abundant mesenchymal tissue with abundant adipose differentiation, and epithelium-lined cysts. Cytogenetic analysis revealed an isolated trisomy 8, as the sole karyotype anomaly, a finding further confirmed by a whole-genome single nucleotide polymorphism array genotyping. The trisomy 8 was observed by fluorescent in situ hybridization within the malformation, and also in adjacent pulmonary parenchyma. A search of the literature revealed only 2 cases having similarities with the present case, but bearing different names. We believe that this lesion differs from congenital pulmonary airway malformations and from adult-type pulmonary hamartomas. We propose for this malformative mass the name "chondroid cystic malformation of the lung."

Management of gastroschisis varies. This study aims to determine which aspects of practice influence outcomes.All cases of simple gastroschisis (N = 99) in the Canadian Pediatric Surgery Network database were analyzed looking at methods of preoperative bowel protection, timing of closure, and closure techniques; and outcome measures included time to onset of enteral feeds, duration of parenteral nutrition (PN), and length of stay (LOS).One third of infants had initial bowel protection using a spring-loaded silo, which was significantly associated with a delay (beyond 24 hours) in establishing primary closure. Neither preoperative bowel protection methods nor defect closure techniques conferred any significant effects on success at establishing primary closure or functional outcomes. After adjusting for all covariates, only failure to establish primary closure was associated with impaired outcomes with significantly delayed initiation of enteral feeds and prolonged LOS. Low birth weight (2000 g) and younger gestational age (36 weeks) were associated with a 3-fold increased risk of longer PN dependence and 5-fold risk of extended LOS, respectively. Babies undergoing the sutureless spontaneous closure technique had significant delays in initiating enteral feeds but no increased requirements for PN or LOS.Modes of preoperative bowel protection and techniques of abdominal wall closure ultimately have no association with functional outcomes in infants with gastroschisis. Failure to establish primary closure, however, is significantly associated with delays in establishing intestinal function and subsequent time to discharge.

AIM: To establish children born with gastroschisis (GS). METHODS: We performed a retrospective study covering the period from January 2000 to December 2007. The following variables were analyzed for each child: Weight, sex, apgar, perforations, atresia, volvulus, bowel lenght, subjective description of perivisceritis, duration of parenteral nutrition, first nasogastric milk feeding, total milk feeding, necrotizing enterocolitis, average period of hospitalization and mortality. For statistical analysis, descriptive data are reported as mean ± standard deviation and median (range). The non parametric test of Mann-Whitney was used. The threshold for statistical significance was P < 0.05 (Two-Tailed). RESULTS: Sixty-eight cases of GS were studied. We found nine cases of perforations, eight of volvulus, 12 of atresia and 49 children with subjective description of perivisceritis (72%). The mortality rate was 12% (eight deaths). Average duration of total parenteral nutrition was 56.7 d (8-950; median: 22), with five cases of necrotizing enterocolitis. Average length of hospitalization for 60 of our patients was 54.7 d (2-370; median: 25.5). The presence of intestinal atresia was the only factor correlated with prolonged parenteral nutrition, delayed total oral milk feeding and longer hospitalization. CONCLUSION: In our study, intestinal atresia was our predictive factor of the severity of GS.

Introduction: The objective of the study was to establish the predictive value of prenatal ultrasound markers for complex gastroschisis (GS) in the first 10 days of life. Material and Methods: In this retrospective cohort study over 11 years (2000-2011) of 117 GS cases, the following prenatal ultrasound signs were analyzed at the last second- and third-trimester ultrasounds: intrauterine growth restriction, intra-abdominal bowel dilatation (IABD) adjusted for gestational age, extra-abdominal bowel dilatation (EABD) ≥25 mm, stomach dilatation, stomach herniation, perturbed mesenteric circulation, absence of bowel lumen and echogenic dilated bowel loops (EDBL). Results: Among 114 live births, 16 newborns had complex GS (14.0%). Death was seen in 16 cases (13.7%): 3 intrauterine fetal deaths, 9 complex GS and 4 simple GS. Second-trimester markers had limited predictive value. Third-trimester IABD, EABD, EDBL, absence of intestinal lumen and perturbed mesenteric circulation were statistically associated with complex GS and death. IABD was able to predict complex GS with a sensitivity of 50%, a specificity of 91%, a positive predictive value of 47% and a negative predictive value of 92%. Discussion: Third-trimester IABD adjusted for gestational age appears to be the prenatal ultrasound marker most strongly associated with adverse outcome in GS.

Recent reviews of gastroschisis identify prematurity and low birth weight as predictors of morbidity and mortality. The authors compared the outcomes of intrauterine growth-restricted infants (IUGR) with gastroschisis to those without growth restriction because IUGR is different from prematurity.A retrospective analysis was performed for infants born with gastroschisis between 1990 and 2000 at 2 pediatric hospitals. Patients were segregated into 3 groups based on birth weight corrected for gestational age: group 1 (IUGR,fifth percentile), group 2 (fifth to 25th percentile), and group 3 (25th percentile). Patient demographics, method of closure, number of surgeries, presence of atresia, and time to full enteral feedings (FPO days) were assessed. Mortality rate, length of stay (LOS), and readmission rates were also compared. Analysis of variance (ANOVA)/Student's t test and Fisher's. Exact tests were used for statistical analysis (P.05 significant). Regression analysis was also performed.One hundred thirteen patients were included (group 1 = 17; group 2 = 43; group 3 = 53). Overall, infants with IUGR had similar outcomes to non-IUGR infants, including FPO and total parenteral nutrition (TPN) days, LOS, readmission, and mortality rates. The method of closure did not affect outcome. Infants with atresia had significantly increased times to full feeding (95 v 34 days; P =.034), more surgeries (2.7 v 1.4; P =.002), and longer LOS (106 v 48 days; P =.011). Infants born at less than 37 weeks' gestation had significantly increased fasting (NPO) days (28 v 18 days; P =.005) and longer LOS (65 v 37 days; P =.006) when compared with infants born at greater than 37 weeks. Logistic regression analysis identified the presence of atresia as an independent risk factor for gastrointestinal dysfunction and the need for prolonged TPN. Prematurity also adversely affected these same parameters, although it did not reach statistical significance.Although infants with gastroschisis are generally small for gestational age, the outcomes of growth-restricted infants are similar to those of other infants. The type of closure does not affect outcome, regardless of birth weight. The presence of atresia or prematurity does lead to longer times for full feeding and LOS. Therefore, routine premature delivery of infants with gastroschisis should not be advocated, even in the context of IUGR.

Background Pediatric ovarian torsion (OT) is a serious condition, especially in cases of asynchronous bilateral ovarian torsion (ABOT). The authors sought to evaluate the predisposing factors for ABOT and to evaluate the most appropriate treatment for ovarian torsion. Methods The authors retrospectively reviewed the charts of patients with ovarian torsion between 1980 and 2002. Data collected included age at presentation, type and duration of symptoms, ultrasound scan findings, interval to surgery, procedures, pathology report, and follow-up. Results Seventy-six patients had adnexal torsion confirmed at surgery, 4 of whom had ABOT. The mean age was 10 years. The mean duration of complaints before hospitalisation and interval to surgery were 56 and 33 hours, respectively. Thirty-five patients had simple tubo-ovarian torsion (46%), including all the patients with ABOT (11.4%), and 41 had an ovarian pathology (54%). All patients with ABOT underwent salpingo-oophorectomy at the first episode. They presented earlier for the second episode and had a shorter interval to surgery where detorsion with oophoropexy was performed. Follow-up ultrasound scan showed perfusion and follicles in the remaining ovary. Conclusions The diagnosis of ovarian torsion often is delayed, especially when a solid tumor is suspected. Conservative management should be strongly considered when there is no underlying ovarian pathology. Furthermore, oophoropexy of the ipsilateral and contralateral ovary should be considered to prevent a potentially devastating recurrence.

The aim of this study was to determine the accuracy of a new computer-assisted stereological technique in obtaining structural information of the lung. We compared the point fraction of lung parenchyma (Pp) and alveolar surface density (Sv) obtained by established manual point/intercept counting methods and compared them with those obtained using a computer-assisted method. Lung tissues obtained from normally grown fetal sheep (n = 6) and from newborn lambs with severe lung hypoplasia (n = 5) were inflation fixed via the trachea and processed for light microscopy. In verification-of-technique experiments, Pp and Sv correlated well with known values. There was a significant linear correlation between manual and computer-assisted stereological measurements for values of Pp (r2 = 0.92) and Sv (r2 = 0.98). Our data lead us to believe that the computer-assisted stereological technique described in this study provides accurate estimates of Pp and Sv and hence may be a valuable tool for evaluating the effects of factors upon structural development of the lung.

Purpose: Pulmonary lymphangiectasia (PL) is a rare, poorly documented disease characterized by abnormal pulmonary lymphatics. Although case reports are published, little is known about survivors past the neonatal period. Methods: This is a retrospective review of histologically proven PL in fetuses, infants, and long term survivors since 1965. Results: Eleven children (8 boys, 3 girls) and 8 aborted fetuses (7 male, 1 female) were identified. The fetuses weighed 463.4 g (177 to 681 g). Six were aborted between 19 to 24 weeks of gestation for multiple malformations or anencephaly, and 2 spontaneously aborted: one with PL only, the other with twin-twin transfusion syndrome. Clinical PL was diagnosed between 0 and 11 months of age. Six children died (2 neonatal, 4 within 10 days), 5 survived. Two deaths occurred after cardiac surgery. Among survivors, the symptomatology and frequency of admissions diminished over time. Symptoms included progressive respiratory distress, chronic cough, recurrent pneumonia, bronchial asthma, and choking. One child with bilateral chylothorax was later diagnosed with Noonan syndrome; 2 patients had minor cardiac malformations. Rapid deterioration occurred with mild respiratory infections with only supportive treatment available. Chest x-ray showed marked hyperinflation with interstitial infiltrate. Conclusions: This is the first long-term study of primary PL and will help counsel parents. Although fatal in the neonatal period, survival is possible if diagnosed past the neonatal period and improvement is expected. J Pediatr Surg 35:796-800. Copyright © 2000 by W.B. Saunders Company.

Iatrogenic Horner syndrome is a rare complication of chest tube insertion, with little information available on this topic in the pediatric literature. We present a case of a 13-month-old boy with a left-sided pneumonia and an associated pleural effusion for which a chest tube was inserted. His respiratory and septic parameters improved, but he was noted to have ptosis, miosis, and anhydrosis of the left side. These resolved in the days after chest tube removal. Although tube thoracostomy is a common procedure in surgical practice, little is written about the potential for injury to the ipsilateral sympathetic chain. This report reviews the available literature, with an emphasis on complication avoidance.

Background Adolescents with a pectus excavatum mostly present with cosmetic complaints and rarely have significant physical limitations. The preoperative evaluation includes pulmonary functions tests, echocardiography, and chest computed tomography (CT) scan to measure the Haller index. In most patients, the chest CT is performed only to measure the Haller index. The purpose of this study was to evaluate whether indices measured on chest radiograph (CXR) and CT scan are comparable. Methods Cases of pectus excavatum treated with the minimally invasive approach in the last year were prospectively collected. In patients for whom a preoperative CXR and CT scan were available, an index was measured using both imaging modalities and compared. Results Both preoperative imaging studies were available in 12 patients. The mean Haller indices on CT scan and CXR were 3.97 and 4.08, respectively. The Pearson correlation score between the 2 groups was 0.984. Conclusions We propose that the Haller index measured on CT scan be replaced by CXR measurement in asymptomatic patients in whom a chest CT scan is otherwise not necessary. This will limit radiation exposure to children. When in doubt, a CT scan of the chest can be used for the preoperative evaluation.

HALOGENATED anesthetic agents have been associated with perioperative liver dysfunction and injury. Most cases of hepatotoxicity reported involve halothane exposure. Halothane produces two types of hepatotoxicity. A mild, transient syndrome with modest increases in transaminase enzymes occurs in 20% of patients given halothane. 1 A more severe and fulminant hepatitis with marked liver dysfunction, jaundice, encephalopathy, and even death is observed in 1 in 20,000 anesthetic exposures to halothane. 2 Halothane toxicity occurs in children as well as in adults.3'4 Cross-sensitization between halothane and other volatile agents has been previously reported with isoflurane,5 enflurane, 6-8 and desflurane. 9 ' 10 To date, only four cases of hepatotoxicity in adults were related to desflurane exposure. 9-12 We report the case of a young child who developed acute hepatotoxicity after desflurane anesthesia in the context of two previous isoflurane exposures.

Congenital pulmonary arteriovenous malformations (AVMs) are rare lesions, usually asymptomatic. We report on the case of a baby who was thought to have a congenital cystic adenomatoid malformation of the left upper lobe based on prenatal and postnatal imaging. Final pathology revealed a congenital pulmonary AVM. Neither the child nor her family have any evidence of hereditary hemorrhagic telangiectasia. To our knowledge, this is the first reported case of a pulmonary AVM mimicking a congenital cystic adenomatoid malformation.

Background/Purpose The optimal mode of delivery for infants with gastroschisis is controversial. The authors compared the outcomes of infants with gastroschisis born vaginally (VD) or by cesarean section (CS). Methods A retrospective analysis of infants with gastroschisis born between 1990 and 2000 was performed. Assessment included patient demographics, respiratory distress, method of closure, number of surgeries, presence of atresia, feeding parameters, parenteral nutrition days (TPN), time to full feeding (FPO), mortality, and length of stay (LOS). Subgroup analyses were performed for those infants requiring cesarean section for fetal distress. Student's t test/analysis of variance (ANOVA) or χ 2 /Fisher's Exact tests were used for statistical analysis. Logistic and linear regression analyses were also performed. Results One hundred thirteen patients were studied (82 VD and 31 CS). No statistical difference existed between the VD and CS groups for perinatal complications, method of closure, number of surgeries (1.6 each), TPN (40.6 v 46.0 days), FPO (40.4 v 47.1 days), mortality (9.7 v 6.5%) and LOS (53.4 v 61.7 days). CS was associated with increased stenosis (25.8 v 4.9%; P=.003), gastrointestinal dysfunction (25.8 v 11.0%; P=.049), and respiratory distress (16.1 v 3.7%; P=.035). Many of these differences did not persist when infants undergoing CS for fetal distress were excluded from the analysis. However, regression analysis identified CS as an independent risk factor for the development of respiratory distress at birth (odds ratio, 7.11; CI, 1.06 to 47.7), with a trend to increased gastrointestinal dysfunction (odds ratio, 4.35; CI, 0.77 to 24.61). Conclusions The routine use of CS for infants with gastroschisis is not supported by our results because equivalent outcomes were observed with both modes of delivery. CS may be a necessary intervention for fetal distress.

Background/Purpose Disease-specific outcome predictors are required for gastroschisis. We derived and validated a gastroschisis prognostic score (GPS) based on bowel appearance after birth. Methods Visual scoring of bowel matting, necrosis, atresia, and perforation generated a novel gastroschisis bowel injury score recorded in a national database. Reweighting of score components by regression analysis led to assessments of model calibration and goodness of fit. The GPS was validated in subsequent cases. Results Records from 225 infants were used for model derivation. Only intestinal necrosis independently predicted mortality by regression analysis (odds ratio, 11.5; 95% confidence interval, 4.2-31.4). Model recalibration identified that a GPS of 4 or more predicted mortality in 75% of nonsurvivors and 99% of survivors ( P = .0001). A GPS of 2 or more demonstrated significantly worse survival outcomes compared with scores of 0 or 1 (length of stay: P = .011, days to first enteral feed: P = .013, days on total parenteral nutrition: P = .006). Model validation with 184 new patients yielded continued high-quality discrimination of outcomes. The GPS demonstrated "near-perfect" interobserver reliability between 2 surgeons ( κ ≥ 0.86). Conclusions The GPS allows the accurate and reliable identification of high-risk groups for mortality and morbidity based on bowel appearance at birth. This information can drive discussions regarding family counseling, resource allocation, and new therapies for these patients.

Background/Purpose The management of asymptomatic congenital lung lesions is controversial. Some centers recommend resection in infancy, and others prefer observation. Our objective was to evaluate the pulmonary function of children who underwent lung resection at 12 months or younger. We hypothesized that these children would not have a significant reduction in pulmonary function when compared with norms for age. Methods All patients at 2 tertiary-care children's hospitals who underwent lung resection at 12 months or younger and are currently older than 5 years were identified and prospectively recruited. Pulmonary function testing was standardized in all patients. Results Fourteen children were tested prospectively, whereas results were available for another 5 children. Four children were excluded for inability to perform pulmonary function testing (n = 2) or for preexisting pulmonary hypoplasia/syndrome (n = 2). Pulmonary function testing values were considered normal if they were more than 80% of predicted. Forced vital capacity was normal in 14 (93%) of 15 children, and forced expiratory volume in 1 second was normal in 13 (86%) of 15 children. Diffusion capacity and respiratory muscle strength were normal in all children tested. Conclusions Most children undergoing lung resection in infancy will have normal pulmonary function tests, supporting our philosophy of early, elective resection of congenital lung lesions.

To describe the outcomes of 130 intestinal atresias between 1982 and 2007. Records were analyzed for location, demographics, prenatal diagnosis, birth weight, associated anomalies, surgery, establishment of oral intake, re-interventions and mortality. Statistical analyses were performed using Fisher test and ANOVA. There were 59 duodenal (30 male), 63 jejuno-ileal (34 male) and 8 colonic atresias (3 male). Prenatal diagnosis was established in 27 (46%) duodenal (DA), 26 (41%) jejuno-ileal (JIA) and 1 (12.5%) colonic atresias (CA). The mean birth weights, 2,380.5 g (SD 988) DA, 2,814 g (SD 755) JIA and 3,153 g (SD 527) CA were significantly different (p = 0.011). The mean gestational ages were 36, 37 and 37 weeks in DA, JIA and CA, respectively (p-NS). Associated congenital anomalies were seen in 41 (76%) DA, 32 (52%) JIA and 3 (38%) CA (p = 0.08, NS). The median time to full oral feeds after surgery was 18 days in DA, 20 days in JIA and 15.6 days in CA, respectively (p > 0.05). Eight patients with DA and nine patients with JIA underwent repeat surgery for adhesive obstruction. Adhesive bowel obstruction was most common in the first year after surgery in both groups (15/17). Gastroschisis was seen in six (10%) of JIA and three (35%) of CA. Two patients in the JIA group underwent bowel lengthening. Patients with gastroschisis and those with associated anomalies needed prolonged duration of TPN after JIA correction. There was no mortality in the duodenal atresia and colonic atresia groups. Six patients in the JIA group died, three of severe atresias coupled with multiple anomalies and three of cholestasis and sepsis. Distal atresias are difficult to diagnose antenatally. Proximal atresias have a significantly lower birth weight than distal atresias. Associated anomaly screening is important in all atresias.

We studied 123 neonates with atresias of the gastrointestinal tract at different levels to assess the role of amniotic fluid in the growth and development of the gastrointestinal tract. Our hypothesis was that significant differences in birth weight between groups otherwise comparable would suggest an intrauterine nutritive role for amniotic fluid. Our second hypothesis was that there is a trophic role of amniotic fluid on intestinal epithelium affecting its functional maturity. Establishing earlier tolerance of enteral nutrition in neonates with distal as compared with proximal atresias would support this hypothesis. Overall, 123 neonates from a single institution, with duodenal atresia (DA), jejuno-ileal (JIA) and colonic atresia (CA), were separated into three groups. Birth weight, gestational age, associated anomalies and duration of total parenteral nutrition intake were recorded. Neonates with multiple atresias and gastroschisis were excluded from analysis. Statistical analysis of differences between groups was carried out using analysis of variance (ANOVA) for independent samples. Post-ANOVA pairwise comparisons were carried out using the Tukey-HSD test. Fifty-four DAs, 62 JIAs and seven CAs were included. The mean birth weight, 2380.5 g (s.d. 988) in DA, 2814 g (s.d. 755) in JIA and 3153 g (s.d. 527) in CA were significantly different between groups (P=0.011). The Tukey-HSD test showed that DA

This paper presents four severe cardiac injuries that occurred in patients who underwent the minimally invasive repair of pectus excavatum (MIRPE). These complications occurred in different clinical settings, namely in a patient with an extremely severe form of pectus, in a patient who had previously undergone an open repair, after a previous open heart surgery, and at the time of bar removal. The purpose of this article is to review the circumstances leading to these cardiac injuries, share what we have learned from these patients, and hopefully help avoid these complications in the future.

Background: Pleuropulmonary blastoma is among the rarest tumors of childhood. Three types have been described: cystic, solid, and mixed. To date, bilateral disease has not been documented. Methods and Results: A 5-week-old girl presented with a history of fever. Chest x-ray showed bilateral diffuse cystic lesions. Bowel obstruction developed that required laparotomy. Multiple small bowel polyps were resected. The patient was readmitted 4 months later with deteriorating respiratory status. The underwent sequential thoracotomies for resection of multiple bullae under high-frequency oscillatory ventilation. Small bowel polypectomies were again required because of obstruction. Lung lesions were compatible with pulmonary blastoma but could not be correlated with intestinal polyposis. Bilateral cystic renal lesions were seen on ultrasound scan. Her disease progressed, despite chemotherapy, with the appearance of metastatic iris lesions. She again underwent laparotomies for multiple recurrent generalized small bowel polyps that were causing obstruction. Expanding renal cysts affected kidney function, and she died at 14 months of age. Conclusions: The rare association between pleuropulmonary blastoma and Wilms' tumor or nephroblastomatosis is known but rarely reported. Lacking pathological evidence, we can only speculate that this was the case. We have been unable to demonstrate any histological association between the renopulmonary and digestive lesions. Despite many unanswered questions, we are likely dealing with a "syndrome" of sorts with a dire outcome, despite aggressive treatments.

The timing and need of resection of asymptomatic congenital lung lesions are controversial. The morbidity of such surgery needs to be considered in the decision analysis. We analyzed the contemporary outcome of infants and neonates undergoing lung resection.With institutional review board approval, all patients 12 months or younger undergoing lung resection between 1995 and 2004 in 2 hospitals were reviewed. Demographic data, indications for surgery, operative procedure, complications, use of regional anesthesia, length of stay (LOS), and follow-up were assessed.Forty-five patients (28 male, 17 female) with a median age of 4 months (2 days-12 months) were evaluated. Congenital lesions (42) were the most frequent indication for surgery. Twenty-two (48.9%) patients had cardiorespiratory symptoms or infection preoperatively. Lobectomy was the most common operation (40/45). Three patients had intraoperative difficulty (bleeding, hypotension, desaturation). Significant postoperative complications occurred in 7 patients: prolonged air leak or chest tube drainage (4), anemia or bleeding (2), respiratory distress requiring reintubation (1). Fewer complications occurred in asymptomatic vs symptomatic patients (1/23 vs 6/22). Of 12 patients, 7 (58%) requiring 24 hours of ventilation or longer were 3 months or younger. Increasing age did significantly influence the chance of successful extubation (P = .01; odds ratio, 1.5; 95% confidence interval, 1.0-2.0), as did the use of epidural anesthesia (P.001). Median LOS was 6 days (2-89 days). Asymptomatic patients had shorter LOS (median, 4 days; range, 2-20 days; P = .024) vs symptomatic patients (median, 8 days; range, 4-89 days). The only death occurred from underlying heart disease. Mean follow-up at 35 months (12-132 months) revealed no subjective reduction in cardiopulmonary function.Lung resection is safe and well tolerated in infancy. Surgery should be scheduled before the development of symptoms but likely after 3 months of age to improve the chances of postoperative extubation. The use of regional anesthesia may facilitate this.

Purpose Outcomes studies for gastroschisis are constrained by small numbers, prolonged accrual, and nonstandardized data collection. The aim of this study is to create a national pediatric surgical network and database for gastroschisis (GS) that tracks cases from diagnosis to hospital discharge. Methods The 16-center network serves a population of 32 million. Gastroschisis cases are ascertained at prenatal diagnosis. Perinatal data include maternal risk and fetal ultrasound variables, delivery plan and outcome, a postnatal bowel injury score, intended and actual surgical treatment, and neonatal outcomes. Institutional review board–approved data collection conforms to regional privacy legislation. Deidentified data are centralized and accessible for research through the network steering committee. Results To date, 114 cases of pre- and/or postnatal gastroschisis have been uploaded. Of 106 live-born infants (40 [38%] by cesarean delivery), 100 had complete records, and overall survival to discharge was 96%, with a mean survivor length of stay (LOS) of 46 days. Infants treated with attempted urgent closure (61%) had significantly shorter LOS (42 vs 57days; P = .048) but comparable LOS compared with those treated with silos and delayed closure. Fetal bowel dilation 18 mm or greater did not predict a difference in outcome. Conclusion Population-based databases allow rapid case accrual and enable studies that should aid in the identification of optimal perinatal treatment.

Background The rarity of pediatric melanoma prompted our review of sentinel lymph node biopsy (SLNB) and associated prognosis. Methods A chart review from 1989 to 2004 revealed 12 cases of cutaneous melanoma. Variables analyzed included demographics, site, histology, tumor characteristics, nodal status, and distant metastasis (TMN status), SLNB and/or therapeutic lymph node dissection (TLND), adjuvant treatment, disease-free survival, and overall survival. Results Mean age at diagnosis was 8.5 years with 7 of 12 patients younger than 10 years (range, 0.3-17.9 years). Site distribution was the extremity (7), trunk (4), and head and neck (1). All patients had wide local excision and primary closure or skin graft. Breslow's thickness averaged 3.5 mm (range, 0.8-6 mm). Only patients diagnosed after 2000 with melanomas thicker than 1 mm were offered SLNB (extremity = 2, trunk = 1, head and neck = 1). Two patients had positive sentinel lymph node: one received TLND and interferon and one is followed closely (unclear pathology). Disease-free survival and overall survival by stage were stage I (n = 2, 3.9 years, 100%), stage II (n = 6, 7.7 years, 83%), stage III (n = 4, 2.6 years, 75%), and stage IV (n = 0). A stage II patient with negative SLNB, adjuvant chemotherapy, and interferon died 26 months after diagnosis, and a stage III patient with clinically and pathologically positive nodes after TLND died 15 months after diagnosis. Conclusion Although a negative SLNB does not guarantee a favorable prognosis, its increasing use will further define its role in pediatric melanoma.

Purpose Thoracoscopy has an expanding role in the treatment of FD cysts (bronchogenic cysts and esophageal duplications). We examined this trend in our patients and reviewed our overall experience. Methods All charts of children undergoing surgery for foregut duplications (FDs) in 2 pediatric hospitals between 1992 and 2003 were retrospectively reviewed. Data gathered included age, weight, symptoms, diagnostic tests, operative technique, postoperative course, complications, and outcome. Results There were 39 children, with FD resected by thoracotomy in 21 patients, thoracoscopy in 11 patients (no conversions to open), cervical incision in 6 patients, and laparotomy in 1 patient for an FD near the gastroesophageal junction. Diagnosis was made by antenatal ultrasound in 7 cases. Four of these neonates had tachypnea or cough, and the rest were asymptomatic. Seventy-five percent of patients diagnosed postnatally presented with respiratory symptoms. Excision of isolated FD (without lung resection) was compared between those who had a thoracotomy (n = 16) vs thoracoscopy (n = 11). The age, weight, operating time, and anesthesia time were not different between the 2 groups. However, the thoracoscopy group had significantly fewer chest tube days (1.6 vs 3.3 days) and a shorter hospital stay (2.6 vs 6.6 days). Intraoperative complications consisted of tracheal injury in 3 patients (2 thoracotomy, 1 thoracoscopy) and esophageal mucosal injury in 2 patients (both thoracotomy), which were all recognized and repaired. Conclusion Foregut duplications may present in a variety of ways and locations. Thoracoscopy is advantageous for isolated intrathoracic FDs.

Background In utero gene transfer may provide advantages for the correction of congenital genetic disorders. In the present study we compare the ability of adenovirus (AdCMVLacZ), and two serotypes of adeno-associated virus (AAVCMVLacZ serotypes 2 and 2/5), to target cardiac and skeletal muscle after prenatal systemic or intramuscular injection in mice and assess the immune response to the vectors. Methods Day 14 gestation fetal mice underwent direct intraperitoneal or intramuscular injection of AdCMVLacZ, and AAVCMVLacZ serotypes 2 and 2/5 vectors. Tissues were processed for β-galactosidase expression in frozen or high-resolution thin plastic sections at early and late time points. Neutralizing antibodies to Ad and AAV were analyzed in separate fetal experimental and neonatal or adult control groups after administration and re-administration of the vectors. Results A single injection of each vector in utero resulted in sustained expression of β-galactosidase transgene in skeletal and cardiac muscle. Transgene expression was detected for the length of the study, i.e. 86, 58, and 31 weeks after birth for AdCMVLacZ, and AAVCMVLacZ serotypes 2 and 2/5, respectively. High-level expression in the myocardium was observed independent of the vector or route of administration. Neutralizing antibody responses to AAV and Ad antigens were reduced and long-term expression in muscle was not ablated on postnatal re-administration of vector. Conclusions Sustained, high-level cardiac and skeletal muscle transgene expression can be obtained after prenatal gene transfer with each of these vectors. The potential for immune response to viral antigens is altered, but not entirely ablated after in utero exposure. Copyright © 2003 John Wiley & Sons, Ltd.

Prolonged fetal tracheal occlusion (TO) accelerates lung growth but leads to loss of alveolar epithelial type II (AE2) cells. In contrast, temporary TO leads to recovery of AE2 cells and their ability to produce surfactant. The aim of this study was to determine the effects of temporary TO in fetal sheep with lung hypoplasia on postnatal lung function, structure, and surfactant protein mRNA expression. Diaphragmatic hernia (DH) was created in 22 fetal sheep at 65 days of gestation. TO was performed between 110 days of gestation and full term (DH/TO, n = 7) and between 110 and 130 days of gestation (DH/TO+R, n = 6). Sham-operated fetuses ( n = 11) served as controls. Lambs were delivered at ∼139 days of gestation, and blood gas tensions were monitored over a 2-h resuscitation period. Temporary TO increased growth of the hypoplastic lung and restored surfactant protein mRNA expression and AE2 cell density but did not improve respiratory function above that of animals that underwent prolonged TO; DH/TO and DH/TO+R lambs were hypoxic and hypercapnic compared with Sham animals. Lung compliance remained low in DH/TO+R lambs, most likely as a consequence of the persistent increase in alveolar wall thickness in these animals.

Free autologous cartilage, which is used in laryngotracheal reconstruction (LTR), may undergo progressive necrosis as a result of delayed revascularization. Angiogenic growth factors, such as vascular endothelial growth factor (VEGF), promote angiogenesis in the ischemic environment. We studied the effect of ex vivo gene transfer of VEGF121 on cartilage angiogenesis and graft survival in a rabbit model of LTR. Sixty rabbits underwent LTR with auricular cartilage. The grafts were treated at 1 × 109 plaque-forming units with 1) VEGF121 (n = 20), 2) LacZ reporter gene (n = 20), or 3) saline solution (n = 20). Graft neovascularization and survival were histologically assayed at 1 and 10 weeks. Angiogenesis was enhanced at both 1 and 10 weeks after treatment with VEGF121 as compared to controls (p < .001). No statistical improvement in graft survival was evident after treatment with VEGF121. Ex vivo gene transfer to cartilage may be a promising gene therapy strategy to enhance revascularization — and, potentially, cartilage survival — under the proper conditions.

Background: The EXIT (ex utero intrapartum treatment) procedure, although initially designed for reversal of tracheal occlusion in fetuses with congenital diaphragmatic hernias (CDH), has been adapted to treat a variety of fetal conditions. Methods: A retrospective chart review of all consecutive EXIT procedures since 1996 was conducted. Results: Thirty-one women underwent the EXIT procedure, with an average maternal age of 29 years (range, 20 to 38), and average gestational age of 34 weeks (range, 29 to 40). The indication was airway obstruction from fetal neck mass in 13, and reversal of tracheal occlusion from in utero clipping in 13. Singular indications included an EXIT-to-ECMO (extracorporeal membrane oxygenation) procedure for a fetus with CDH and a cardiac defect (n = 1), congenital high airway obstruction syndrome (CHAOS, n=1), resection of a very large congenital cystic adenomatoid malformation of the lung (CCAM) on uteroplacental bypass (n = 1), unilateral pulmonary agenesis (n = 1), and thoracoomphalopagus conjoined twins. The mean duration on uteroplacental bypass (from uterine incision to umbilical cord clamping) was 30.3 [plusmn] 14.7 minutes (range, 8 to 66). No fetus experienced hemodynamic instability during uteroplacental bypass as recorded by fetal heart rate (FHR), pulse oximeter, and fetal echocardiography, except for one instance of reversible bradycardia from umbilical cord compression. The mean FHR and fetal saturation were 153.0 [plusmn] 38.5 beats per minute and 71.2% [plusmn] 19.9%, respectively. Five fetuses required a tracheostomy. Only 1 death occurred during an EXIT procedure because of inability to secure the airway secondary to extensive involvement by a lymphangioma. The average cord pH and pCO 2 were, respectively, 7.20 [plusmn] 0.11 and 63.2 [plusmn] 14.6. Two maternal complications occurred: bleeding from a hysterotomy site and dehiscence of an old hysterotomy scar noticed at a subsequent cesarean section. The average maternal blood loss was 848.3 [plusmn] 574.1 mL. Conclusion: The EXIT procedure was used successfully to ensure uteroplacental gas exchange and fetal hemodynamic stability during a variety of surgical procedures performed to secure the fetal airway or ensure successful transition to postnatal environment.

In this study we investigate the efficacy of lentiviral vectors of different pseudotypes for gene transfer to tissues of the preimmune fetus. BALB/c fetuses at 14-15 days' gestation received lentiviral vectors carrying the transgene lacZ under the control of the human cytomegalovirus (CMV) promoter by intramuscular (i.m.) or intrahepatic (i.h.) injection. We pseudotyped the lentiviral vectors with vesicular stomatitis virus (VSV-G), with Mokola virus, or with Ebola virus envelope glycoproteins. We harvested the pups at time points between 5 days and 9 months following injection and performed a detailed histologic assessment. The efficiency and distribution of transduction after in utero administration was highly dependent upon the route of administration and the pseudotype of vector used. Biodistribution studies showed widespread distribution of vector sequences in multiple tissues, albeit at very low levels, and transduced cells were found in significant numbers only in liver, heart, and muscle. Overall, VSV-G was the most efficient in transducing hepatocytes, whereas Mokola and Ebola were more efficient in transducing myocytes. Transduction of cardiomyocytes was observed after both i.m. and i.h. injection of all three vectors. Our findings of long-term transduction of skeletal myocytes and cardiomyocytes after in utero administration suggest a novel strategy for the treatment of congenital muscular dystrophies.

Background/Purpose: Increased neuroproliferation in the appendix associated with an increase in substance P (SP), vasoactive intestinal polypeptide (VIP), and growth-associated protein-43 (GAP-43) has been documented in appendices of adults with acute right lower quadrant (RLQ) abdominal pain and absence of gross or histologic signs of appendiceal inflammation. The authors tested whether these findings were present in children with RLQ pain and a normal appendix. Methods: Immunohistochemistry staining of paraffin-embedded appendices was performed with GAP-43, VIP, and SP. The positive control group included appendices with acute inflammation (group I, n=5); the negative control group included appendices removed incidentally (group II, n=5); and the experimental group included appendices from children suspected to have acute appendicitis without histologic signs of inflammation (group III, n=9). Results: Group I: VIP was strongly expressed in the nerve plexuses. The lamina propria and muscularis showed absent or minimal VIP expression. SP staining was strong in all plexuses and was moderate to strong in the muscularis. SP expression in the epithelium and lamina propria was difficult to quantify secondary to inflammation. Group II: VIP expression was essentially undetectable in the epithelium, lamina propria, and muscularis, and was moderate in the nerve plexuses. Mild SP staining was detected in the nerve plexuses of most specimens, and absent to mild staining was found in the epithelium and muscularis. However, one specimen strongly expressed SP in all layers. Group III: VIP expression was moderate to strong in the lamina propria and muscularis of nearly all specimens, and strong expression was found in all nerve plexuses. All but one specimen strongly expressed SP in plexuses. There was moderate to strong expression of SP in the epithelium, lamina propria, and muscularis in over 50% of specimens. The immunostaining for GAP-43 was very weak and nonspecific and did not help discriminate between the 3 study groups. Conclusions: Increased neuroproliferation in the lamina propria and muscularis was evident in patients with abdominal pain and normal appendices compared with appendices removed incidentally. The VIP and SP expression in these patients was similar or higher than that observed in patients with acute inflammation on histology. J Pediatr Surg 36:1222-1226. Copyright © 2001 by W.B. Saunders Company.

Two patients presented as full-term baby girls with anorectal and genital malformations with extensive perineal hemangiomas. The first patient had a vestibular anus with a perineal hemangioma involving the bladder, rectal, and vaginal walls. Skin ulcerations required a transverse loop colostomy for wound care. The vulva, urethral opening, and clitoris were deviated to the left, labia minora were absent, and the labia majora were abnormal. The second patient had an anus displaced anteriorly and deviated to the right. The external anal sphincter was hypertrophic on the left and atrophic on the right. Rectal examination showed agenesis of the right levator ani and a dentate line located at the skin level. She had a large perineal, sacral, vaginal, pararectal and retroperitoneal hemangioma and developed extensive skin ulcerations. She had only a hemiclitoris located to the left of the midline, near absence of labia minora, and hypertrophied labia majora. The urethra was displaced to the left and opened in the vestibule. Both patients had a spinal malformation (one with tethered cord and one with spina bifida) and a normal karyotype. Steroids and interferon allowed near-complete resolution of hemangiomas in both patients. The authors were impressed by the similarity of these two cases and could not find any previous description of this association.