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Purpose: We describe our series of giant omphalocele patients treated with a serial taping method for gradual reduction of the abdominal contents and early fascial closure., Methods: Between 2010 and 2017 we cared for ten newborns with giant omphaloceles. The average gestational age was 35.5 weeks (range 29-38) and average birthweight was 2.84 kg. Seven infants had other major anomalies, including one with a variant of Pentology of Cantrell. Four had abnormal chromosomes. None had any attempt to primarily close the defect. Omphalocele defects were serially taped at bedside in the NICU with the child awake until the viscera were completely reduced, and the defect could be closed., Results: Mean time to closure was 13.7 days (median 14 days). Six were closed primarily without a patch. The remaining four infants required Gore-Tex patch (covered by skin) which was later removed and fascia closed in three infants (at 70 days, 75 days, and 11 months of age). Total length of stay was a mean 71.8 days (median 71)., Conclusions: Serial taping achieves early fascial closure and avoids complications of a staged surgical approach, such as multiple anesthetics, loss of fascial margin integrity, silo dehiscence, and fistula formation. Compression of the viscera is slow enough to avoid abdominal compartment syndrome and the fascia and amnion are left intact leaving the option available to use escharotic agents if required., Type of Study: Treatment study., Level of Evidence: Level III., (Copyright © 2018 Elsevier Inc. All rights reserved.)

Background: In our fetal lamb model of lower urinary tract obstruction, a pressure limited shunt preserves bladder function and renal development. This study investigates the effects on pulmonary histology., Methods: We created obstructive uropathy (OU) in 60-day gestation fetal lambs, ligating the urethra and urachus, and delivering them at term (130-145days). We compared pulmonary histology in 4 groups: group A, OU without shunt; group B, pressure limited shunt; group C, non-valved shunt. Shunts were inserted 3weeks post-obstruction. Group D were normal controls., Results: We compared 27 fetuses: 7 fetuses in group A, 4 fetuses in group B, 2 fetuses in group C, and 14 fetuses in group D. There was no significant difference in lung volume in any group. In group A, there were some areas of atelectasis and incomplete alveolar formation histologically. The alveoli in group A lambs lungs had a predominance of type II cells, whereas group B lambs lungs were lined by type I epithelial cells and were essentially indistinguishable from controls., Conclusions: This study suggests that using a pressure-limited vesico-amniotic shunt in OU may preserve the renal tract and the lungs., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Purpose: Hydrops and pulmonary hypoplasia are associated with significant morbidity and mortality in the setting of a congenital lung lesion or pleural effusion (PE). We reviewed our experience using in utero thoracoamniotic shunts (TA) to manage fetuses with these diagnoses., Methods: A retrospective review of fetuses diagnosed with a congenital lung lesion or pleural effusion who underwent TA shunt placement from 1998-2013 was performed., Results: Ninety-seven shunts were placed in 75 fetuses. Average gestational age (±SD) at shunt placement and birth was 25±3 and 34±5 weeks. Shunt placement resulted in a 55±21% decrease in macrocystic lung lesion volume and complete or partial drainage of the PE in 29% and 71% of fetuses. 69% of fetuses presented with hydrops, which resolved following shunt placement in 83%. Survival was 68%, which correlated with GA at birth, % reduction in lesion size, unilateral pleural effusions, and hydrops resolution. Surviving infants had prolonged NICU courses and often required either surgical resection or tube thoracostomy in the perinatal period., Conclusion: TA shunts provide a therapeutic option for select fetuses with large macrocystic lung lesions or PEs at risk for hydrops and/or pulmonary hypoplasia. Survival following shunting depends on GA at birth, reduction in mass size, and hydrops resolution., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Background: In our fetal lamb model of lower urinary tract obstruction, a valved shunt preserves bladder function. This study investigates the effects on renal histology., Methods: We created obstructive uropathy (OU) in 60-day gestation fetal lambs, ligating the urethra and urachus, and delivering them at term (130-145days). We compared renal histology in 4 groups: group A-OU without shunt, group B-low-pressure shunt (15-54mmH2O), group C-high-pressure shunt (95-150mmH2O). Shunts were inserted 3weeks post-obstruction. Group D were normal controls., Results: We delivered 32 fetuses from 23 ewes: 13 fetuses in group A (9 survived), 6 fetuses in group B (5 survived), 7 fetuses in group C (5 survived), and 6 fetuses in group D. Histologically, we found renal tubular distention, vacuolated degeneration of tubular epithelial cells in 7 lambs, and cyst formation in 4 lambs in group A. There was renal tubular distention in two lambs, and cyst formation in one lamb in both groups B and C, with vacuolated degeneration of tubular epithelial cells observed in all but 1 lamb in each group., Conclusions: V-A shunting prevents multicystic dysplastic kidney (MCDK). Some lambs have renal tubular distention and vacuolated degeneration of renal tubular epithelial cells., (Copyright © 2014 Elsevier Inc. All rights reserved.)

Background/purpose: Severe neonatal pulmonary hypoplasia incurs mortality rates approaching 71% to 95%. We sought to determine the utility of serial amnioinfusions through a subcutaneously implanted intraamniotic catheter to prevent pulmonary hypoplasia in fetal obstructive uropathy., Methods: Fetal lambs (n = 32) were divided into 3 groups. Group I (n = 12) underwent a sham operation, group II (n = 15) underwent a complete urinary tract obstruction via ligation of the urachus and urethra with a subcutaneous tunneled intraamniotic port-a-cath without amnioinfusions, and group III (n = 5) underwent a creation of a complete urinary tract obstruction with a port-a-cath as described in group II with serial amnioinfusions. Lung tissue was analyzed by lung volume to body weight ratios and stereology. Statistical analysis was performed by analysis of variance and Bonferroni comparisons (P < .05)., Results: Obstructed fetuses grossly had smaller lungs than treated and control animals. Lung volume to body weight ratios were statistically significant between groups. Airspace fractions were comparable between groups I and III (average = 0.53 and 0.55, respectively), although both were significantly greater than group II (average = 0.48) (P = .049)., Conclusions: Serial amnioinfusions through an intraamniotic port-a-cath prevented pulmonary hypoplasia in an ovine model of complete obstructive uropathy. The use of an easily accessible device for amnioinfusions may be a viable option to treat oligohydramnios., (Copyright © 2011 Elsevier Inc. All rights reserved.)

Introduction: We previously demonstrated that in utero vesicoamniotic shunting of obstructive uropathy in fetal lambs produces a shrunken noncompliant bladder. We hypothesized that the normal fetal bladder filling and emptying cycle in fetal life is critical to the development of normal bladder function., Materials and Methods: We placed vesicoamniotic shunts in 4 normal fetal lambs at 74 days' gestation. The fetuses were delivered at term (145 days), and bladder volume and compliance were measured and compared with those measurements in 3 normal term fetuses. The lambs were then killed and the renal tracts and bladders removed submitted to histologic examination., Results: All shunted lambs survived to term. Three normal control lambs were delivered at term. The mean bladder volume in shunted lambs was 4 +/- 2.8 mL (n = 4) compared with 60 +/- 17 mL (n = 3) in control lambs (P < .05). Bladders in the shunted lambs had very poor compliance compared with normal lambs' bladders. Histologic examination of the shunted bladders showed increased fibrosis and distortion of the muscle layers compared with control bladders., Conclusion: Even in the absence of obstruction, preventing normal bladder filling and emptying in fetal life produces fibrotic bladders with poor compliance.

Introduction: In utero shunting (vesico-amniotic shunt) of obstructive uropathy in fetal lambs produces a shrunken, noncompliant bladder. We hypothesized that using a ventriculo-peritoneal shunt for the vesico-amniotic shunt may preserve the filling/emptying cycle and thus normal bladder development., Materials and Methods: We created obstructive uropathy in 60-day gestation fetal lambs, ligating the urethra and urachus. Vesico-amniotic shunting was performed 21 days later using the valve end of a ventriculo-peritoneal shunt (valve shunt) or silastic tubing (nonvalve shunt). They were delivered at term (145 days), and the bladder volume was measured and compared to normal term fetuses. The lambs were sacrificed, and the kidneys and bladder removed for histology., Results: Twenty-seven lambs were shunted. Of 14 valve shunts, 8 were effective. Of 13 nonvalve shunts, 11 were effective. The mean bladder volume was 57 +/- 41 mL with a valve shunt and 8.8 +/- 4.7 mL with a nonvalve shunt (P < .05) (normal term lambs, 65 +/- 18 mL, n = 5). Histology of the shunted bladders showed increased fibrosis in the submucosal and muscle layers. This was less obvious in lambs with a valve shunt., Conclusion: A pressure controlled shunt for fetal obstructive uropathy improves bladder volume but does not prevent bladder wall fibrosis.

Purpose: The long-term outcome for children after antenatal intervention for obstructive uropathies is disappointing. We reported that renal dysplastic changes are well established 3 weeks after obstruction in a fetal lamb model. We used this model to explore renal development and bladder function after fetal intervention., Methods: We created an obstructive uropathy in fetal lambs at 60 days gestation by ligating the urethra and urachus. A vesicostomy (female) or urethrostomy (male) were performed 21 days later. The fetuses were killed at term (145 days) and bladder volume and compliance were measured. The urinary tract was processed for histologic examination., Results: Twenty two fetuses were shunted. Nine were miscarried or were still-born. Thirteen survived, and 11 had a successful shunt with a small bladder (8 +/- 5 mL) compared with controls (71 +/- 19 mL) (P < .05). Shunted bladders had poor compliance. Histologically, they had thickened submucosal connective tissue with hypertrophied muscle. Histology of the renal tissue demonstrated relatively well-preserved renal architecture with reduced nephron mass (oligonephronia) in 2 lambs and multicystic dysplastic change in 3. Six (55%) had normal nephrogenesis., Conclusions: In our model, shunt operations after obstructive uropathy fail to preserve bladder function. Shunting ameliorated the development of cystic dysplasia, but half of the lambs had oligonephronia or multicystic dysplastic kidney. These might develop renal failure later in life.

This is a report of our experience with 22 cases of large unruptured omphaloceles treated by amnion inversion during the period 1973 through 1990. The method is characterized by three stages: (1) a silastic sheet is sutured directly to the skin around the amniotic membrane, under local anaesthesia, without dissection between the skin and the amnion; (2) the reduction of herniated viscera into the abdominal cavity is achieved by squeezing the sheeting using a specially modified stapler; and (3) the amniotic membrane is preserved intact, and inverted into the abdominal cavity at the time of abdominal wall closure. Of the 22 infants, 19 survived with satisfactory results. Two patients died of multiple associated anomalies, and the remaining patient died of sepsis arising at the time of the final abdominal closure. This procedure has proved to be effective and safe for high-risk patients with congenital heart diseases, anal atresia, tracheoesophageal fistula, or bronchial stenosis and prematurity. The practical aspects of the procedure, as well as its advantages and pitfalls, are illustrated.

Operative repair of giant omphaloceles remains a technical challenge to close the wide abdominal wall defect. Currently, most surgeons remove the amnion to approximate the linea alba and/or skin edges or to suture prosthetic material to the abdominal wall and cover the defect with skin flaps. In doing so, the liver commonly becomes extruded and distended with blood, compounding the problem of reducing all of the viscera within the small abdominal cavity. Furthermore, bowel obstruction from adhesions produced from opening the abdomen is a life-long threat. We describe six cases of giant omphalocele in which the amnion was left intact, and it was progressively inverted into the abdominal cavity by using the silastic silo, as it is used for gastroschisis. The birth weight of these infants ranged from 2,360 to 3,240 g. The abdominal wall defect measured 7.0 cm to 10.5 cm in width, and protruded at least 8 cm beyond the abdominal wall. The first stage of repair was to suture the silastic silo to the skin-amnion junction, and progressively reduce the bowel and liver within the abdomen. The intrabdominal pressure is monitored by nasogastric tube or by an indwelling bladder catheter to avoid pressures greater than 20 cm H2O, which might compromise intestinal and renal circulation. The second stage consisted of incising the skin/amnion junction to expose the linea alba. The linea alba was approximated while leaving the amnion intact and folding it into the abdominal cavity. This avoids entering the peritoneum or interfering with the blood flow to and from the liver.(ABSTRACT TRUNCATED AT 250 WORDS)

The ideal surgical closure for large omphaloceles remains controversial. Most techniques described advocate removal of the amniotic sac prior to repair of the abdominal wall defect. Herein, the authors describe a surgical technique preserving the amniotic sac, which has distinct advantages in achieving safe and rapid closure of the omphalocele by primary or secondary means in selected patients.