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Background: There are two approaches to close gastroschisis. Primary closure (PC) is reduction and fascial closure; silo closure (SC) places viscera in a preformed-silo and reduces the contents over time. We have shifted from PC to SC. This study compared the outcomes of these two techniques. Methods: Records of babies with gastroschisis from 1994-2004 were reviewed. Closure type, ventilator days, days to full-feeds, hospital days, complications, and mortality were recorded. Results: Twenty-eight patients underwent PC; 20 patients had SC. Differences in ventilator days, days to full-feeds, and hospital days were not statistically significant. Nine PC patients developed closure-related complications vs. none in SC (P < 0.05). Eight PC vs. two SC patients had non-closure-related complications (P < 0.05). Four PC vs. zero SC patients developed necrotizing enterocolitis (P < 0.05). Five PC vs. one SC patients had ventral hernia (P < 0.05). No patient died. Conclusion: PC resulted in higher incidence of reclosure, non-closure-related complications, and necrotizing enterocolitis. Consequently, we recommend SC as the preferred treatment. [ABSTRACT FROM AUTHOR]

Cholecystectomy may benefit children with biliary colic without stones on ultrasound (US) or low ejection fraction on cholecystokinin-hepatobiliary iminodiacetic acid (CCK-HIDA) scan. Children with symptomatic biliary colic and abnormal HIDA scan, specifically those with high ejection fractions, may benefit from cholecystectomy. All patients younger than 18 years old undergoing cholecystectomy from 2008 to 2012 in our practice were reviewed. Patients with a negative US and CCK-HIDA ejection fractions 80 per cent or greater were included in the study. Patient data were extracted from charts, whereas postoperative symptoms were obtained by phone interviews. Of 174 patients who underwent cholecystectomy, 12 (7%) met study criteria. All patients (12 of 12) had evidence of cholecystitis on the final pathology note. All 11 patients contacted had relief of colic after gallbladder removal with a mean follow-up of 16 months. A subset of pediatric patients with high ejection fractions on CCK-HIDA and symptomatic biliary colic may have symptomatic relief with cholecystectomy.

Zenker's Diverticulum (ZD) is a false diverticulum located in the posterior esophagus between the cricopharyngeus and inferior constrictor. Few cases have been reported in the pediatric population. Congenital esophageal diverticulum is commonly associated with additional esophageal anatomical deformities or iatrogenic injury in the early post-natal period. TD was born at 39 5/7 weeks gestational age and did well at home with the exception of "choking during feeds 2-4 times per day" with mild cyanosis. Flexible bronchoscopy performed under sedation revealed a retropharyngeal mass protruding towards the epiglottis resulting in upper airway obstruction. Esophogram demonstrated upper esophageal diverticulum. Patient underwent left neck dissection with cervical myotomy from the cricopharyngeus to the level of the thoracic inlet. A postoperative repeat esophogram illustrated resolution of the diverticulum. Currently, the patient is 9 months postoperative and is tolerating feeds by mouth, gaining weight and has no pulmonary symptoms. There have been a few reports of ZD in children occurring in combination with anatomical abnormalities or esophageal manipulation. This case is one of the youngest reported and occurs in a patient without any previous manipulation of the esophagus. This condition is exceedingly rare but should be included in the differential diagnosis for both pharyngoesophageal and tracheal symptoms., (Published by Elsevier Inc.)

Background: Chronic pancreatitis requiring surgery is rare in children. We review our experience in treating pediatric chronic pancreatitis with longitudinal pancreaticojejunostomy (LPJ)., Methods: Records of children with chronic pancreatitis treated with LPJ between 1997 and 2003 were reviewed. Demographic data, associated conditions, endoscopic interventions, operative procedures, postoperative complications, length and costs of hospitalization, and long-term outcome were recorded., Results: Four patients (one girl), 3 to 16 years old, underwent LPJ. Associated conditions included bile duct obstruction (2), single (1) or multiple (1) pancreatic duct strictures, recurrent familial pancreatitis (1), pseudocyst (1), Down's syndrome (1), and duodenal web (1). Preoperative endoscopic stenting was performed in two patients. All were on restricted diets, one on parenteral nutrition. Pre-LPJ, each child had 3 to 6 admissions for pancreatitis with mean total cost of 39,000 dollars, excluding diet charges. At surgery, two patients required biliary diversion for persistent biliary obstruction in addition to LPJ. Postoperatively, no patient developed fistulas or anastomotic leaks. There were no deaths. The median length of hospitalization post-LPJ was 8 days with mean cost of US37,000 dollars. All patients resumed a normal diet post-LPJ. There were no recurrences of pancreatitis with follow-ups between 2 and 6 years., Conclusion: Longitudinal pancreaticojejunostomy is safe and cost-effective for treating pediatric chronic pancreatitis. It has minimal complications and frees patients from pancreatitis-related hospitalizations.

Purpose: To determine the interobserver variability of prenatal magnetic resonance (MR) lung volumetry and to assess the value of MR lung volumetric findings as predictors of outcome in fetuses with congenital diaphragmatic hernia., Materials and Methods: Prenatal MR imaging was performed in 26 fetuses with unilateral congenital diaphragmatic hernia. Two independent observers performed planimetric measurement of lung volume. Relative lung volume was calculated as the observed total lung volume expressed as a percentage of the total lung volume predicted from fetal size. Relative lung volume was correlated with the ultrasonographic lung-head ratio in left-sided congenital diaphragmatic hernias evaluated before 27 weeks gestation (n = 21) and with pregnancy outcome in all cases of isolated left-sided congenital diaphragmatic hernia without prenatal intervention (n = 11)., Results: Observers demonstrated excellent agreement in total lung volume measurements at MR imaging, with an intraclass correlation coefficient of 0.95. Relative lung volume was positively correlated with lung-head ratio (r = 0.78, P <.001). By using rank order analysis in the pregnancy outcome group, relative lung volume was predictive of prognosis (P <.05) when adjusted for gestational age at delivery and birth weight. Three of four fetuses with a relative lung volume of less than 40% died., Conclusion: Interobserver agreement is high at MR lung volumetry, and its findings are predictive of outcome in fetuses with isolated left-sided congenital diaphragmatic hernia.

Purpose: To determine which parameters are most closely correlated with normal fetal total lung volume and to investigate the use of these parameters in the evaluation of fetal pulmonary hypoplasia., Materials and Methods: Single-shot rapid acquisition with relaxation enhancement (RARE) magnetic resonance (MR) imaging was used to perform planimetric measurement of total lung volume in 46 fetuses at 18-32 weeks gestation. Total lung volume was correlated with gestational age, and biometric parameters in fetuses were correlated with normal chest findings at ultrasonography (US) (n = 24). This analysis was used to evaluate relative lung volume in fetuses suspected of having pulmonary hypoplasia (n = 22)., Results: Normal fetal total lung volume was strongly correlated with liver volume measured at MR imaging (r = 0.94), fetal weight estimated at US (r = 0.93), head circumference measured at US (r = 0.90), and gestational age (r = 0.87). In fetuses suspected of having pulmonary hypoplasia, the relative lung volume varied from 4.6% to 81.6% when the observed total lung volume was expressed as a percentage of the predicted total lung volume., Conclusion: Normal fetal total lung volume is strongly correlated with biometric measurements. Relative fetal lung volume can be calculated by expressing the observed volume as a percentage of the predicted volume calculated from biometric measurements; knowledge of the relative fetal lung volume assists in the confirmation and quantification of fetal pulmonary hypoplasia.

Background/purpose: A variety of techniques have been used to manage pediatric congenital tracheal stenosis. The authors report the technique of slide tracheoplasty for a child with long congenital tracheal stenosis., Methods: A 2-year-old male presented with a history of stridor with feeding. Bronchoscopy findings showed 50% stenosis from complete cartilaginous rings, extending from 2.5 cm below the vocal cords to 2 cm above the carina. Through a neck incision, the trachea was exposed from the cricoid to both bronchi and transected at the midpoint of the stenosis. The upper trachea was split anteriorly to the area of stenosis just below the cricoid. The lower trachea was split posteriorly in the midline. Posterior dissection allowed sliding and anastomosis of both tracheal segments while the lateral vascular supply was left intact. A brace was placed to maintain cervical flexion, and the patient underwent extubation in the operating room., Results: He recovered without complication and was dis charged on postoperative day 4., Conclusion: Slide tracheoplasty offers several advantages for tracheal reconstruction because it is performed with the native tracheal tissues, can be accomplished through a transverse collar incision, and can repair long stenoses without significant tracheal shortening.

Background: A subset of fetuses with sacrococcygeal teratoma (SCT) develops hydrops caused by high-output heart failure. Identification of fetuses at risk for hydrops is important because fetal intervention may reverse the pathophysiology of the disease. To date, no reliable sonographic prognostic factors have been identified., Methods: An experienced sonologist reviewed the sonographic records of 17 fetuses with SCT referred to the authors' institution. Size of the tumor was measured and corrected for fetal size. The appearance of the tumor (solid versus cystic) and its vascularity were graded on a subjective scale of 1 to 5., Results: Only 4 of 12 fetuses that had hydrops survived; of the survivors, 3 had undergone fetal intervention. All nonhydropic fetuses survived. Fetuses with hydrops had tumors that were mainly solid and highly vascular, whereas nonhydropic fetuses had predominantly cystic tumors with comparatively less vascularity. There was no significant difference in tumor size between these 2 groups., Conclusions: Fetuses with SCT that are mainly solid in appearance and are highly vascularized have a higher risk of getting hydrops in utero. Tumor size is not an independent prognostic factor.

Successful open repair of a cleft lip in utero has the advantage of scarless wound healing in the fetus. Unfortunately, no long-term outcome studies have been performed to evaluate the efficacy of these repairs. Moreover, no study to date has compared the long-term results of an in utero cleft lip repair to a similar, control-matched, newborn cleft repair. This study was performed to evaluate the 9-month outcome of in utero cleft lip surgery compared with an identical cleft lip repair performed on infant lambs. In utero epithelialized cleft lips were created through an open hysterotomy in sixteen 65-day-old fetal lambs (term = 140 days) using methods described by Longaker et al. Eight of 16 animals underwent subsequent in utero repair of these clefts at 90 days gestational age. The repair of the remaining eight animals was delayed until 1 week postpartum. At 9 months, the animals were analyzed for changes in lip contour and for the degree of scarring by hematoxylin and eosin and Masson's trichrome collagen staining. Two animals in each group died from preterm labor. Of the animals that survived to term, all repaired lips had some degree of abnormality postoperatively. One of six lips repaired in utero dehisced before delivery. Three of six neonatal repairs dehisced in the first postoperative month. In the remaining animals with intact lip repairs, the vertical lip height on the repaired side was an average of 9 to 12 mm shorter than the normal lip in both the in utero and neonatally repaired animals. Phenotypically, the postnatally repaired animals had more lip distortion and visible notching. Histologically, the in utero repair was scarless and the neonatal repairs had scar throughout the entire vertical height of the lip with an associated loss of hair in this region. Maxillary growth was also evaluated. There was no inhibition of maxillary growth in the animals that underwent in utero cleft lip repair. However, in the neonatal repair group, significant maxillary retrusion was evident. Compared with the cleft side of the maxilla, horizontal growth was decreased by 11 percent (p = 0.01). Compared with the intrauterine repair group, there was a 17-percent decrease in horizontal maxillary width (p = 0.01). Straight-line in utero repair of a cleft lip produces a better long-term result in terms of maxillary growth than a similar repair performed postnatally in the ovine model. There was no diminution in maxillary growth in the animals treated in utero. Histologically, in utero repair of clefts was indeed scarless. However, both lip repairs produced lips that were significantly shorter than their contralateral noncleft sides. This degree of lip shortening would require a secondary lip revision, thereby defeating the purpose of performing an intrauterine repair. Comparisons now need to be made between in utero and neonatal repairs using a Millard-type rotation advancement technique before intrauterine treatment can be considered to be more beneficial than our current treatment modalities.

Purpose: The aim of this study was to examine the feasibility, safety, and short-term outcome of complete one-stage repair of high imperforate anus in the newborn boy., Methods: A retrospective review was conducted of five full-term male infants who underwent posterior sagittal anorectoplasty without a colostomy within the first 48 hours of birth. Preoperative imaging was performed to assess associated anomalies. All infants underwent cystoscopy before the perineal operation to determine the level of the urinary tract fistula, if present. After completion of the anoplasty, all were turned supine and the colon irrigated free of meconium. Follow-up ranged from 10 to 24 months., Results: Laparotomy was not required for any patient. Three patients had a rectoprostatic urethral fistula, one a rectovesical fistula, and one no fistula (common wall at level of prostate). Tapering rectoplasty was required for only the one patient with a rectovesical fistula. There were no intraoperative complications. All patients passed stool within 12 hours after operation and took full feeding by 48 hours. The average hospital stay was 7 days. Postoperative and stenosis occurred in one patient secondary to parental noncompliance with the postoperative dilation regimen. There were no perineal wound complications. All patients have a strong urinary stream and defecate spontaneously without the aid of oral medication or rectal stimulation or enemas., Conclusions: One-stage repair of high imperforate anus in the male neonate is feasible without short-term genitourinary or gastrointestinal morbidity. Whether it is preferable compared with a delayed (two or three stage) repair depends on ultimate long-term anorectal function, which cannot be assessed for several years.

The prospect of fetal surgery for cleft lip is predicated on our ability to accurately identify fetuses with clefts and exclude those that have associated anomalies. Prenatal ultrasound is currently the most appropriate means with which to do this. We reviewed the ultrasonographic data from two large perinatal referral institutions to determine the natural history of fetuses with cleft lip who may be candidates for fetal surgery. Forty fetuses had a cleft lip diagnosed prenatally by ultrasound. In this group, severe associated anomalies were common (30 of the 40) and multiple (23 of the 40) in a majority of fetuses. Life-threatening anomalies, such as central nervous system and cardiac anomalies, were the most common defects. As a result, many fetuses were aborted therapeutically or died in the perinatal period. Out of 12 surviving fetuses, only six had isolated clefts, and two surviving fetuses, diagnosed with isolated cleft lip, had no defect identified postnatally. This information has important implications for the perinatal management of fetuses with cleft lip and the potential role of fetal intervention.

Background/purpose: Snakebite envenomation is a potentially life-threatening form of trauma, the dangers of which are amplified in children because their smaller size increases the relative dose of venom received. The authors reviewed a large series of snakebitten children to address the medical and fiscal issues of treating these patients., Methods: The records of 37 snakebitten children (1987 through 1997) were analyzed for demographic data, signs of envenomation, use of specific therapies (antivenin, blood products, or surgery), length of hospitalization, complications, and cost of care., Results: Fifty-four percent of the children had a major envenomation demonstrated by systemic symptomatology, laboratory analysis, or need for surgery. All children made full recoveries with most receiving only supportive care (92%). The average time to emergency department presentation was 8 hours, where all children with major envenomations and those requiring specific therapies (surgery, clotting factors) were identified. Cost analysis showed an average of $2,450 dollars per child with the majority of expenses attributable to length of hospitalization., Conclusions: Most snakebitten children completely recover with minimal supportive care, and they can be cared for safely and cost effectively as outpatients if no signs of major envenomation are noted within 8 hours of the bite.

The purpose of this study was to determine the effectiveness of intrauterine reconstruction of lateral facial clefts caused by surgically simulated amniotic band compression. The authors hypothesized that intrauterine lysis of these bands could: 1) prevent the progression of cleft formation; 2) normalize facial development; and 3) allow for the scarless repair of these lateral atypical facial clefts. In a prospective randomized trial, eight 65-day gestational fetal lambs (term, 140 days) were exposed via a maternal hysterotomy. A Tessier 7 facial cleft was made by applying a 2.0 nylon suture as a constriction band to the growing face. The sutures were attached to the zygomatic arch and looped circumferentially into the oral commissure. Two weeks after surgery, four of eight animals were re-exposed. All four animals developed phenotypic changes consistent with Tessier 7 lateral facial clefts. These animals were treated in utero by 1) excising the synthetic constricting band, and 2) opening the epithelialized edges of the lateral facial clefts and performing a primary repair. At birth, the bands on the four untreated animals were cut. The lambs developed to 3 months of age, at which time they were evaluated anthropomorphically for changes in soft tissue measurements, and histologically using hematoxylin and eosin and Mason trichrome stain for the degree of scar at the repair site. Tessier 7 lateral facial clefts were produced in all the untreated animals. Each developed macrostomia with an average 2.3-cm lateral displacement of the oral commissure and partial maxillary clefting induced by the pressure of the restriction band. No evidence of tissue necrosis, maceration, or ulceration was noted. In contrast, animals treated in utero were phenotypically normal. There was no evidence of macrostomia and all anthropomorphic measurements were symmetrical. No bony clefts were seen on gross examination. Histologically, there was no evidence of scar formation at the site of the cleft repair. These findings suggest that like the standard cleft lip, atypical lateral facial clefts can be repaired successfully in utero without scarring. Furthermore, lysis of a restriction band in utero can prevent the progression of this facial deformity, leading to normal development of the face.