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Importance: There is variability in practice and imaging usage to diagnose cervical spine injury (CSI) following blunt trauma in pediatric patients., Objective: To develop a prediction model to guide imaging usage and to identify trends in imaging and to evaluate the PEDSPINE model., Design, Setting, and Participants: This cohort study included pediatric patients (<3 years years) following blunt trauma between January 2007 and July 2017. Of 22 centers in PEDSPINE, 15 centers, comprising level 1 and 2 stand-alone pediatric hospitals, level 1 and 2 pediatric hospitals within an adult hospital, and level 1 adult hospitals, were included. Patients who died prior to obtaining cervical spine imaging were excluded. Descriptive analysis was performed to describe the population, use of imaging, and injury patterns. PEDSPINE model validation was performed. A new algorithm was derived using clinical criteria and formulation of a multiclass classification problem. Analysis took place from January to October 2022., Exposure: Blunt trauma., Main Outcomes and Measures: Primary outcome was CSI. The primary and secondary objectives were predetermined., Results: The current study, PEDSPINE II, included 9389 patients, of which 128 (1.36%) had CSI, twice the rate in PEDSPINE (0.66%). The mean (SD) age was 1.3 (0.9) years; and 70 patients (54.7%) were male. Overall, 7113 children (80%) underwent cervical spine imaging, compared with 7882 (63%) in PEDSPINE. Several candidate models were fitted for the multiclass classification problem. After comparative analysis, the multinomial regression model was chosen with one-vs-rest area under the curve (AUC) of 0.903 (95% CI, 0.836-0.943) and was able to discriminate between bony and ligamentous injury. PEDSPINE and PEDSPINE II models' ability to identify CSI were compared. In predicting the presence of any injury, PEDSPINE II obtained a one-vs-rest AUC of 0.885 (95% CI, 0.804-0.934), outperforming the PEDSPINE score (AUC, 0.845; 95% CI, 0.769-0.915)., Conclusion and Relevance: This study found wide clinical variability in the evaluation of pediatric trauma patients with increased use of cervical spine imaging. This has implications of increased cost, increased radiation exposure, and a potential for overdiagnosis. This prediction tool could help to decrease the use of imaging, aid in clinical decision-making, and decrease hospital resource use and cost.

Thyroid surgery in children results from three main etiologies: Medullary thyroid cancer in MEN syndromes, benign disease most often Graves' disease, and thyroid nodules which may harbor differentiated thyroid cancers. I will discuss the evaluation of these etiologies, pre-operative preparation, and operative strategies for each of these pediatric thyroid problems., (Copyright © 2023 Elsevier Inc. All rights reserved.)

Introduction: Von Hippel-Lindau disease (VHL) is a rare cause of hereditary bilateral Pheochromocytomas (PHEO). Traditionally, treatment has been total adrenalectomy due to a lifetime risk of developing new tumors. Limited data exists on the surgical management of bilateral PHEO in children with VHL. We reviewed our experience with laparoscopic partial adrenalectomy for bilateral PHEO., Methods: A retrospective review was performed of patients undergoing adrenalectomy for PHEO in children with VHL from 2004 to 2019., Results: Eight children with VHL diagnosed with bilateral PHEO underwent 16 adrenalectomies (10 synchronous, 5 metachronous, 1 for recurrence). Median age at diagnosis was 13 [range 8-17] years with a median tumor size of 2.3 [range 0.5-7.7] cm. Of 16 adrenalectomies, all were performed laparoscopically, 14 were partial adrenalectomies; 2 patients required a contralateral total adrenalectomy due to size and diffuse multinodularity. There were no postoperative complications. No patients required corticosteroid replacement at the end of the study period. Two patients had new ipsilateral tumors identified after a median follow up of 5 [range 4-6] years with one undergoing repeat partial adrenalectomy. There were no mortalities in the study period., Conclusion: Partial adrenalectomy for bilateral PHEO in patients with VHL is safe and does not compromise outcomes. When technically feasible, laparoscopic partial adrenalectomy should be considered as a primary surgical approach for children with VHL., Level of Evidence: Level IV - Case series with no comparison group., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Background: Hypocalcemia occurs frequently after a total thyroidectomy in pediatric patients. Four hour postoperative PTH monitoring predicts the need for calcium supplementation in the adult thyroidectomy population. We evaluated the role of the 4 h postoperative PTH level in determining the need for calcium supplementation after thyroidectomy in the pediatric population., Methods: This is a retrospective review of children undergoing total thyroidectomy by a single pediatric surgeon from July 2011 through July 2018. Intact PTH obtained four hours postoperatively determined the need for calcium supplementation for patients beginning in November 2014 onward. Serum total calcium levels were monitored concurrently with serum intact PTH levels. Serum calcium levels were followed in our Multispecialty Pediatric Endocrine Surgery clinic within the month following thyroidectomy., Results: From July 2011 through July 2018, there were a total of 56 total thyroidectomies at our institution. Prior to November 2014, all pediatric total thyroidectomies received calcium supplementation per our institutional protocol. Based on ionized calcium levels, 26.3% (5/19) of children developed hypocalcemia. From November 2014 to July 2018, 37 pediatric patients required total thyroidectomies. 29.7% (11/37) had low 4-h postoperative PTH levels. 72.7% (8/11) patients with low 4-h postoperative PTH levels had corresponding postoperative day 1 total calcium levels less than 8.5 or ionized calcium levels less than 1.12, and five children (45.5%) developed symptomatic hypocalcemia. 70% (26/37) of children had normal 4-h postop PTH levels, with only 5 (19%) ever developing hypocalcemia. No patients with a normal postop PTH level developed symptomatic hypocalcemia or required IV calcium repletion. A single 4-h postoperative PTH <10 pg/dl for identifying hypocalcemia has a sensitivity of 81% and specificity of 91%, with AUC 0.81., Conclusion: The 4-h postoperative serum PTH level can help determine the need for calcium supplementation in pediatric patients undergoing total thyroidectomy, thereby reducing unnecessary calcium supplementation and serial lab draws to monitor for postoperative hypocalcemia., Level of Evidence: Level II., (Copyright © 2019 Elsevier Inc. All rights reserved.)

Background: Omphaloceles can be some of the more challenging cases managed by pediatric surgeons. Single center studies have not been meaningful in delineating outcomes due to the length of time required to accumulate a large enough series with historical changes in management negating the results. The purpose of this study was to evaluate factors impacting the morbidity and mortality of neonates with omphaloceles., Methods: A multicenter, retrospective observational study was performed for live born neonates with omphalocele between 2005 and 2013 at nine centers in the United States. Maternal and neonatal data were collected for each case. In-hospital management and outcomes were also reported and compared between neonates with small and large omphaloceles., Results: Two hundred seventy-four neonates with omphalocele were identified. The majority were delivered by cesarean section with a median gestational age of 37 weeks. Overall survival to hospital discharge was 81%. The presence of an associated anomaly was common, with cardiac abnormalities being the most frequent. Large omphaloceles had a significantly longer hospital and ICU length of stay, time on ventilator, number of tracheostomies, time on total parenteral nutrition, and time to full feeds, compared to small omphaloceles. Birth weight and defect size were independent predictors of survival., Conclusion: This is the largest contemporary study of neonates with omphalocele. Increased defect size is an independent predictor of neonatal morbidity and mortality., Level of Evidence: Level II., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: Perforated appendicitis can be managed with early appendectomy, or nonoperative management followed by interval appendectomy. We aimed to identify the strategy with the lowest health care utilization and cost., Methods: We retrospectively reviewed the medical records of all children ≤18 years old with perforated appendicitis admitted to a single institution between January 2009 and March 2016. After excluding immunosuppressed patients and transfers from outside hospitals, we grouped the remaining patients by early or interval appendectomy. Cost accounting data were obtained from our institutional database. The primary outcome was total hospital cost over 2 y from initial admission for appendicitis. Other outcomes analyzed included initial admission costs, number of admissions, emergency room and clinic visits, percutaneous procedures, cross-sectional and overall imaging studies, and length of stay., Results: A total of 203 children with perforated appendicitis were identified. After exclusion of immunosuppressed patients and outside hospital transfers, 94 patients were included in the study. Thirty-nine underwent early appendectomy and 55 initial nonoperative management; of these, 54 underwent elective interval appendectomy. Five of 55 patients (9%) failed initial nonoperative management and required earlier-than-planned appendectomy. Total cost over 2 y was significantly lower with early appendectomy than initial nonoperative management ($19,300 ± 14,300 versus $26,000 ± 17,500; P = 0.05). Early appendectomy resulted in fewer hospital admissions, clinic visits, invasive procedures, and imaging studies., Conclusions: Early appendectomy results in lower hospital costs and less health care utilization compared with initial nonoperative management with elective interval appendectomy. A prospective study will shed more light on this question and can assess the role of nonoperative management without interval appendectomy in children with perforated appendicitis., (Copyright © 2017 Elsevier Inc. All rights reserved.)

Introduction: Preoperative determination of perforated versus acute appendicitis can be difficult. We compared CT and MRI performance in diagnosing perforated appendicitis, and created diagnostic criteria., Methods: We retrospectively reviewed all pediatric patients who underwent appendectomy within one day of CT or MRI between 1/1/2013 and 1/16/2016. True diagnosis was determined by pathology report. Findings on CT/MRI were grouped into "hard" findings (abscess, pneumoperitoneum, extruded fecalith, appendiceal wall with visible hole) and "soft" findings (extensive/diffuse inflammation/free fluid, phlegmon). Correlation of white blood cell count (WBC), temperature, peritoneal signs, and symptom duration >72 h with perforation was assessed using logistic regression. Significant correlates were incorporated in clinical criteria., Results: 135 patients underwent appendectomy after CT/MRI. Fifty patients underwent MRI and 85 CT. Using hard and/or soft findings, MRI was 86.7% sensitive and 74.3% specific, compared to 68.4% (p = 0.19) and 92.4% (p = 0.025) for CT. WBC > 15, temperature >38.0 °C, and peritoneal signs predicted perforation. Diagnostic accuracy of MRI was highest using imaging findings alone. Accuracy of CT was improved by mandating at least one of the previous three clinical correlates, resulting in 68.4% sensitivity and 93.9% specificity., Conclusions: MRI trended toward more sensitive and CT was more specific for complicated appendicitis. CT specificity is improved by our algorithm.

Background: The role of US-guided fine-needle aspiration biopsy (US-FNAB) of thyroid nodules is not well-established in children., Objective: To retrospectively assess the utility of US-FNAB of pediatric thyroid nodules., Materials and Methods: We reviewed Department of Radiology records to identify children who underwent US-FNAB of the thyroid between 2005 and 2013. Two board-certified pediatric radiologists reviewed pre-procedural thyroid US exams and documented findings by consensus. We recorded cytopathology findings and compared them to surgical pathology diagnoses if the nodule was resected. We also recorded demographic information, use of sedation or general anesthesia, and presence of on-site cytopathological feedback. The Student's t-test was used to compare continuous data; the Fisher exact test was used to compare proportions., Results: US-FNAB was conducted on a total of 86 thyroid nodules in 70 children; 56 were girls (80%). Seventy-eight of the 86 (90.7%) US-FNAB procedures were diagnostic; 69/78 (88.5%) diagnostic specimens were benign (including six indeterminate follicular lesions that were proved at surgery to be benign) and 9/78 (11.5%) were malignant/suspicious for malignancy (all proved to be papillary carcinomas). There was no difference in size of benign vs. malignant lesions (P = 0.82) or diagnostic vs. non-diagnostic lesions (P = 0.87). Gender (P = 0.19), use of sedation/general anesthesia (P = 0.99), and presence of onsite cytopathological feedback (P = 0.99) did not affect diagnostic adequacy. Microcalcifications (P < 0.0001; odds ratio [OR] = 113.7) and coarse calcifications (P = 0.03; OR = 19.4) were associated with malignancy. Diagnoses at cytopathology and surgical pathology were concordant in 27/29 (93.1%) nodules; no US-FNAB procedure yielded false-positive or false-negative results for malignancy., Conclusion: US-FNAB of pediatric thyroid nodules is feasible, allows diagnostic cytopathological evaluation, and correlates with surgical pathology results in resected nodules.

The current diagnostic accuracy and perinatal outcome of fetuses with esophageal atresia (EA) continues to be debated. In this review, we report on our experience at a tertiary care fetal center with the prenatal ultrasound diagnosis of EA. Enrollment criteria included a small/absent stomach bubble with a normal or elevated amniotic fluid index between 2005 and 2013. Perinatal outcomes were analyzed and compared to postnatally diagnosed EA cases. Of the 22 fetuses evaluated, polyhydramnios occurred in 73%. Three (14%) died in utero or shortly after birth, but none had EA. In the presence of an absent/small stomach and polyhydramnios, the positive predictive value for EA was 67%. In fetal EA cases confirmed postnatally (group 1, n = 11), there were no differences in gestational age, birthweight, or mortality when compared to postnatally diagnosed infants (group 2, n = 59). Group 1 was associated with long-gap EA, need for esophageal replacement, and increased hospital length of stay. When taken in context with the current literature, we conclude that ultrasound findings suggestive of EA continue to be associated with a relatively high rate of false positives. However, among postnatally confirmed cases, there is an increased risk for long-gap EA and prolonged hospitalization.

Purpose: A major determinant of survival in patients with congenital diaphragmatic hernia (CDH) is severity of pulmonary hypoplasia. This study addresses the comparative effectiveness of prenatal methods of lung assessment in predicting mortality, extracorporeal membrane oxygenation (ECMO), and ventilator dependency., Methods: We retrospectively reviewed all patients born with isolated CDH between 2004 and 2008. Lung-to-head ratio (LHR) and observed-to-expected LHR (OELHR) were obtained from prenatal ultrasounds. Percent-predicted lung volume (PPLV) was obtained from fetal MRI (fMRI). Postnatal data included in-hospital mortality, need for ECMO, and ventilator dependency at day-of-life 30., Results: Thirty-seven patients underwent 81 prenatal ultrasounds, while 26 of this sub-cohort underwent fMRI. Gestational age during imaging study was associated with LHR (p=0.02), but not OELHR (p=0.12) or PPLV (p=0.72). PPLV, min-LHR, and min-OELHR were each associated with mortality (p=0.03, p=0.02, p=0.01), ECMO (p<0.01, p<0.01, p=0.03), and ventilator dependency (p<0.01, p<0.01, p=0.02). For each outcome, PPLV was a more discriminative measure, based on Akaike's information criterion. Using longitudinal analysis techniques for patients with multiple ultrasounds, OELHR remained associated with mortality (p=0.04), ECMO (p=0.03), and ventilator dependency (p=0.02), while LHR was associated with ECMO (p=0.01) and ventilator dependency (p=0.02) but not mortality (p=0.06)., Conclusion: When assessing fetuses with CDH, OELHR and PPLV may be most helpful for counseling regarding postnatal outcomes., (Copyright © 2013 Elsevier Inc. All rights reserved.)

Background: Recurrent herniation is a well-known complication following the initial repair of congenital diaphragmatic hernias (CDHs). The role of minimally invasive surgical techniques in recurrent CDH remains undefined. The purpose of this study was to evaluate our early experience with thoracoscopic repair compared with traditional open repair in children with recurrent CDH., Subjects and Methods: We retrospectively reviewed all recurrent Bochdalek CDH cases (n=24) managed at a single tertiary-care referral center between January 1990 and March 2011. Children who underwent thoracoscopic repair for recurrent CDH were identified, and their data were compared by the unpaired t test and the two-sided Fisher's exact test, as appropriate, with those of children who underwent open repair. Significance was defined as P<.05., Results: Thoracoscopic repair was attempted in 6 (25%) children with recurrent CDH. Four (67%) repairs were successfully completed without conversion to an open procedure. The mean age at thoracoscopic repair was 11.5 months (range, 8.1-16.1 months). The mean operative time was 191 minutes (range, 94-296 minutes), and all children were extubated within 24 hours. The mean hospital length of stay was 3.75 days (range, 1-6 days). There were no deaths or subsequent recurrences after a mean follow-up of 26.5 months (range, 14.3-41.3 months). There were no statistical differences in any of the measured outcome variables when compared with the open repair group., Conclusions: Our initial experience suggests that thoracoscopic repair is a feasible alternative to open repair in selected children with recurrent Bochdalek diaphragmatic hernias.

Purpose: This review provides a blueprint to deal with the diagnosis and management of recurrent tracheoesophageal fistulas., Methods: A retrospective review over 27 years found 26 recurrent tracheoesophageal fistulas. Descriptive statistical analyses were performed., Results: In this cohort of 26 patients, 18 had a leak after their primary operation; and 22 had respiratory symptoms leading to the discovery of the recurrent fistula. The diagnosis was made by contrast study in 24. The repairs entailed placing a catheter through the fistula; separating the trachea and esophagus using sharp dissection; and placing tissue, preferably pericardium, between the suture lines. Postoperative complications included 7 anastamotic leaks, 4 strictures, and 3 recurrent fistulas. Long-term follow-up (median of 84 months) showed that 21 took all of their nutrition by mouth, 3 were tube fed, and 2 required a combination. Of the 23 patients with growth chart data, 16 fell in the first quartile of the growth chart, whereas none fell between the 75th and 100th percentile., Conclusion: This series, the largest to date, describes characteristics of recurrent tracheoesophageal fistulas, including techniques to make the diagnosis and provide a secure closure of the fistula, and the long-term outcomes of these patients., (Copyright 2010. Published by Elsevier Inc.)

Purpose: Anorectal malformations (ARMs) are associated with a large number of functional sequale that may affect a child's long-term quality of life (QOL). The purposes of this study were to better quantify patient functional stooling outcome and to identify how these outcomes related to the QOL in patients with high imperforate anus., Methods: Forty-eight patients from 2 children's hospitals underwent scoring of stooling after 4 years of life. Scoring consisted of a 13-item questionnaire to assess long-term stooling habits (score range: 0-30, worst to best). These results were then correlated with a QOL survey as judged by a parent or guardian., Result: Mean (SD) age at survey was 6.5 (1.6) years. Comparison of QOL and clinical scoring showed no signficant difference between the 2 institutions (P > .05). There was a direct correlation between the QOL and stooling score (Pearson r(2) = 0.827; beta coefficient = 24.7, P < .001). Interestingly, functional stooling scores worsened with increasing age (Pearson r(2) = 0.318, P = .02). Patients with associated congenital anomalies had a high rate of poor QOL (44% in poor range; P = .001). Stooling scores decreased significantly with increasing severity/complexity of the ARM (P = .001)., Conclusion: A large number of children experience functional stooling problems, and these were directly associated with poor QOL. In contrast to previous perceptions, our study showed that stooling patterns are perceived to worsen with age. This suggests that children with ARMs need long-term follow-up and counseling., (Copyright 2010 Elsevier Inc. All rights reserved.)

Background: Cervical spine clearance in the very young child is challenging. Radiographic imaging to diagnose cervical spine injuries (CSI) even in the absence of clinical findings is common, raising concerns about radiation exposure and imaging-related complications. We examined whether simple clinical criteria can be used to safely rule out CSI in patients younger than 3 years., Methods: The trauma registries from 22 level I or II trauma centers were reviewed for the 10-year period (January 1995 to January 2005). Blunt trauma patients younger than 3 years were identified. The measured outcome was CSI. Independent predictors of CSI were identified by univariate and multivariate analysis. A weighted score was calculated by assigning 1, 2, or 3 points to each independent predictor according to its magnitude of effect. The score was established on two thirds of the population and validated using the remaining one third., Results: Of 12,537 patients younger than 3 years, CSI was identified in 83 patients (0.66%), eight had spinal cord injury. Four independent predictors of CSI were identified: Glasgow Coma Score <14, GCSEYE = 1, motor vehicle crash, and age 2 years or older. A score of <2 had a negative predictive value of 99.93% in ruling out CSI. A total of 8,707 patients (69.5% of all patients) had a score of <2 and were eligible for cervical spine clearance without imaging. There were no missed CSI in this study., Conclusions: CSI in patients younger than 3 years is uncommon. Four simple clinical predictors can be used in conjunction to the physical examination to substantially reduce the use of radiographic imaging in this patient population.

Purpose: The purpose of the study was to assess the treatment strategies and outcome of right-sided congenital diaphragmatic hernia (R-CDH), particularly extracorporeal membrane oxygenation (ECMO)., Methods: We reviewed the cases of 42 patients treated for R-CDH at our institution from 1991 to 2006. We gathered demographic information, documented ECMO use and the type of surgical repair, and compared outcomes with predicted survival as calculated by the CDH Study Group's equation., Results: Of the 35 patients included in our statistical analysis (7 were excluded), 12 (34%) were born at our institution, all of whom were prenatally diagnosed with R-CDH. Nineteen patients (54%) required ECMO therapy. Extracorporeal membrane oxygenation was initiated after repair of the R-CDH in 2 patients (11%). Of those patients who went on ECMO before repair, 4 patients (21%) were repaired on ECMO, 9 patients (47%) underwent repair after ECMO, and 4 patients (21%) underwent ECMO but died before their R-CDH could be repaired. Primary repair of the diaphragm was possible in 15 cases (56%), and primary closure of the abdominal incision was possible in 15 of the 23 open repairs (65%). The mean predicted survival for all 35 patients was 63%, whereas 28 (80%) actually survived. Logistic regression showed a significant association between the presence of cardiac defects and mortality (odds ratio = 0.008, P = .014)., Conclusions: Our data suggest that patients with R-CDH have high ECMO utilization and may experience greater relative benefit from ECMO as evidenced by their higher-than-expected overall survival. Extracorporeal membrane oxygenation may be found to have a distinctive role in managing R-CDH. More high-powered series are needed to elucidate differences between R-CDH and left-sided CDH that may dictate alternate forms of management.

Background/purpose: An increasing number of children are diagnosed with gallstones today. The best management of nonpigmented gallstones in children without hematologic disorders is not known., Methods: The authors prospectively studied 74 children with cholelithiasis diagnosed with ultrasonography. Clinical presentation, natural history, complications, and indications for cholecystectomy were examined. The follow-up (mean, 21 months) consisted of routine clinic visits, chart reviews, and telephone questionnaires with the children or their parents., Results: Of the 74 children, 33 required cholecystectomies, and 41 were followed. The average age was similar in the 2 groups (11.7 v 11.0 years). Children with risk factors for cholelithiasis required earlier surgical treatment (P < .001). In the operative group, 8 presented acutely and 25 electively. There were 2 complications, a wound infection and a retained common duct stone. In the group that underwent follow-up, 34 of 41 children remained asymptomatic or had symptoms improve with dietary manipulation. No complications developed during the follow-up period., Conclusions: Children with gallstones and typical symptoms of right upper quadrant or epigastric pain with food intolerance should undergo cholecystectomy. Eighty-two percent of children with cholelithiasis and atypical symptoms had improvement with dietary manipulation. Pediatric patients with gallstones that are asymptomatic or associated with atypical symptoms can be safely followed without complications.

Although the majority of reported pericardial teratomas have been excised successfully in the postnatal period, the outcome of prenatally diagnosed pericardial teratomas remains less favorable. Two recent cases of prenatally diagnosed pericardial teratomas and a review of previously reported cases suggest a new management algorithm for those pericardial teratomas discovered in utero. Fetuses in whom hydrops does not develop may be safely followed up and treated postnatally. However, if hydrops develops, the fetus requires treatment with either aspiration of the pericardial effusion, or fetal surgery and resection.

Background: Cardiac procedures in exteriorized fetuses or assisted by fetoscopy require monitoring capabilities not attended by conventional maternal transabdominal echocardiography., Methods: We, therefore, assessed the potential of fetal transesophageal echocardiography (TEE) utilizing an intravascular ultrasound catheter (IVUC) for fetal cardiac monitoring. We inserted a 10-F-10-MHz IVUC into the esophagus in 12 exteriorized fetal sheep and by a fetoscopic approach in 4 fetal sheep. Cardiac events were observed. Heart rate, cardiac rhythm, patency of the foramen ovale and ductus arteriosus, and the width of the branch pulmonary arteries could be assessed in all fetuses. Ventricular contractility could be assessed only in fetuses weighing less than 2.5 kg. Larger fetuses did not allow adequate imaging of the apical portion of the ventricles because of limited tissue penetration of the IVUC. Fetal TEE permitted placing small guide wires in the cardiac atria and left ventricle. Short-lived premature beats following intracardiac manipulations of these wires could be observed by fetal TEE in all cases., Results: At autopsy, no complications from IVUC insertion were observed in the exteriorized fetuses. Fetoscopic placement of the IVUC resulted in minor perioral skin erosion in two nonexteriorized fetuses., Conclusions: In conclusion, fetal TEE can be achieved with minor fetal injury and may provide useful information during open and fetoscopic cardiac procedures. Further improvements in IVUC design will permit the application of this technique to monitor human fetal cardiac procedures.

The goal of treatment for penile agenesis is early female gender assignment and feminizing reconstruction of the perineum. Historically, this required multiple operations including both vesicostomy and colostomy. The present case demonstrates the feasibility of early total reconstruction through a posterior sagittal approach that avoids diversion of stool and urine.

In gastroschisis, the eviscerated fetal bowel frequently is damaged and this results in hypoperistalsis and malabsorption. The mechanistic link that ties gastroschisis-induced intestinal damage to dysfunction may be nitric oxide (NO) and the enzyme responsible for producing it, NO synthase. Using a fetal rabbit model, the authors investigated the hypothesis that the hypoperistalsis and malabsorption associated with gastroschisis may be attributable to abnormal small bowel NO synthase activity. Using the 3H-arginine-to-3H-citrulline conversion assay, they measured NO synthase activity in the small bowel of full-term fetal rabbits with and without gastroschisis. The mean total small bowel NO synthase activity of fetal rabbits with gastroschisis was 2.5 times greater than that of control littermates without gastroschisis (n = 6; 5,726 +/- 834 v 2,208 +/- 537 mean pmol/mg protein/min; P = .004). This increased NO synthase activity also was studied by measuring the individual isoforms of NO synthase, and the site of increased NO synthase activity was localized to the small bowel epithelium and neurons. After detecting and localizing the gastroschisis-induced increase in NO synthase activity, the authors explored the mechanism of this increase using NADPH-diaphorase staining. With this histological staining technique, no quantitative increase was found in the small bowel NO synthase of the rabbits with gastroschisis. This suggests that the increased NO synthase activity found in these rabbits is the result of accelerated enzyme kinetics. These findings suggest that the increased NO synthase activity caused by gastroschisis may contribute to the common clinical sequelae of malabsorption and intestinal dysmotility.

Fetal surgery can correct several life-threatening malformations before birth. Despite recent advances in fetal surgery, preterm labor remains a major problem directly related to the large uterine incision required for fetal exposure. Fetal endoscopic surgery ("Fetendo") obviates the need for a large uterine incision and may reduce the overall risks of fetal surgery by causing less uterine trauma and ultimately less preterm labor. Temporary tracheal occlusion is a promising strategy to enlarge the lungs in fetuses with congenital diaphragmatic hernia. Using the technology developed for laparoscopic surgery and for temporary tracheal occlusion, we have developed an endoscopic technique for tracheal occlusion with an endoscopic clip in a fetal sheep model. The evolution of this technique may allow temporary tracheal occlusion without incisional hysterotomy or maternal laparotomy.

As presently understood, cloacal exstrophy results from a migration failure of the lateral mesodermal folds of the infraumbilical anterior abdominal wall, and rupture of the resulting enlarged, persistent cloacal membrane before the eighth week of gestation. The authors present ultrasonographic evidence that disputes this embryological theory. Routine ultrasonography of a twin gestation at 18 weeks showed that one twin had a dilated cloacal abnormality, bilateral hydronephrosis, and oligohydramnios. Repeat ultrasonography at 24 weeks demonstrated rupture of the cloacal anomaly, with resolution of both the hydronephrosis and oligohydramnios. This twin was born with classic cloacal exstrophy. This striking ultrasound evidence of an intact cloacal membrane at 18 weeks, which ruptured before 24 weeks, relieving the urinary tract outlet obstruction, forces us to rethink how this surgically correctable anomaly develops.

In utero repair of selected life-threatening malformations in the human fetus is now a clinical reality, yet fetal surgery continues to pose significant risks to both the mother and the unborn child. Preterm labor is a major problem directly related to the large uterine incision required for fetal exposure. Using technology developed for laparoscopic surgery, we have devised instruments and techniques to perform fetal endoscopic surgery. We now report a percutaneous technique for direct endoscopic access to the uterus. Minimally invasive fetoscopic surgery may expand the indications for fetal surgery by decreasing fetal risks, facilitating intervention earlier in gestation, and reducing preterm labor. This technique was developed in 4 fetal lambs who underwent endoscopic intervention at 105-110 days gestation (term = 145 days). Under ultrasound guidance, a 20-gauge spinal needle was advanced through the maternal abdomen, uterus, and directly into the amniotic cavity. Warmed saline was infused through the needle to expand the amniotic cavity. Next, a 5-mm balloon-tipped trocar was placed percutaneously with ultrasound guidance into the amniotic cavity. A 5-mm laparoscope was introduced and under endoamniotic vision two more 5-mm trocars were percutaneously placed. In all four sheep a 5-mm trocar was placed percutaneously into the gravid uterus. The most difficult step was puncturing through the amniotic membranes, but the sharp tip of the trocar facilitated getting into the amniotic cavity. Excellent visualization of the fetus was obtained with minimal uterine trauma. We have developed a fetoscopic technique in sheep for percutaneous placement of trocars into the uterus using ultrasound guidance. This approach allowed excellent visualization of the fetus with significantly less uterine trauma than open fetal surgery and is an essential prerequisite for future fetal endoscopic interventions.