Tumor volume to fetal weight ratio as an early prognostic classification for fetal sacrococcygeal teratoma

Purpose

This study was designed to develop a prognostic factor for fetuses with sacrococcygeal teratoma (SCT) that may be useful to predict outcome and guide counseling early in pregnancy. We hypothesize that, in fetuses with SCT, the ratio of tumor size to estimated fetal weight in the second trimester predicts outcome.

Methods

We retrospectively reviewed charts of all patients evaluated at our Fetal Center for SCT between 2004 and 2009. Estimated fetal weight and tumor volume were calculated based on prenatal ultrasound or fetal magnetic resonance imaging. Patients were stratified based on tumor volume to fetal weight ratio (TFR), and their outcomes were analyzed by Fisher's Exact test.

Results

Tumor volume to fetal weight ratio before 24 weeks' gestation was predictive of outcome. Those with a TFR less than or equal to 0.12 (n = 5) had a significantly better outcome than patients with a TFR greater than 0.12 (n = 5, P < .05). All patients with poor outcomes had a TFR greater than 0.12 by 24 weeks' gestation. A TFR greater than 0.12 predicted poor outcome with 100% sensitivity and 83% specificity. All 4 patients who developed hydrops had a TFR greater than 0.12.

Conclusion

In our series of fetuses with SCT, TFR before 24 weeks' gestation correlates with outcome. This novel, prenatal diagnostic tool may be useful in prenatal counseling and for early identification of high-risk fetuses.     

Predicting the severity of congenital high airway obstruction syndrome

Background

Congenital high airway obstruction syndrome (CHAOS) is caused by complete or near-complete obstruction of the fetal airway. Obstruction sets in motion a sequence of events that can ultimately lead to fetal demise. However, on rare occasions in utero airway decompression occurs, reversing syndromic findings and improving the prognosis. In our relatively large series of CHAOS patients, we have observed a spectrum of clinical severity. The aim of this study was to identify the prenatal characteristics of CHAOS predictive of a milder postnatal course.

Methods

The medical charts of all fetuses observed at our institution with the diagnosis of CHAOS were reviewed for radiologic findings, delivery information, perinatal course, autopsy or discharge report, and long-term follow-up.

Results

Between 1996 and 2008, 12 fetuses with CHAOS were identified. Four fetuses had no evidence of hydrops on initial imaging. Of the 8 fetuses displaying hydrops, 3 were terminated, 2 died in utero, and 1 with multiple anomalies died at birth. Six fetuses were delivered via the ex utero intrapartum therapy procedure for attempted salvage, and 5 of the 6 survived the neonatal period including all 4 fetuses without hydrops. Serial prenatal imaging demonstrated less severe signs of CHAOS in 3 fetuses, and in 2 of them, direct laryngoscopy revealed a tiny opening in the airway. All 3 fetuses that showed improvement on serial imaging survived the neonatal period and were discharged home by 2-10 weeks of age.

Conclusions

Although the natural history of CHAOS is variable, trends in prenatal ultrasound findings are highly predictive of postnatal outcome and are a valuable guide to prenatal counseling.     

Prenatal diagnosis and outcome of fetal lung masses

Aim

The purpose of this study is to evaluate the accuracy of prenatal diagnostic features, particularly congenital cystic adenomatoid malformation volume ratio (CVR), in predicting outcomes for fetuses with lung masses.

Methods

The records and imaging features of all fetuses referred to the Texas Children's Fetal Center with a fetal lung mass between July 2001 and May 2010 were reviewed retrospectively. Data collected included gestational age (GA) at diagnosis, fetal magnetic resonance imaging findings, CVR, mass size, nature of fetal treatment, surgical findings, pathology, and outcome. Data were analyzed for predicting development of hydrops or the need for fetal therapy using receiver operating characteristic curves.

Results

Of 82 fetuses (41 male) evaluated for a lung mass, 53 (65%) were left-sided (1 bilateral), and the mean (SD) GA at diagnosis was 21.5 (4.3) weeks. Seventy-three fetuses underwent fetal magnetic resonance imaging at a mean (SD) GA of 26.1 (4.6) weeks. Thirteen fetuses (16%) had fetal treatment. Four fetuses with hydrops underwent open fetal surgical resection, and 3 survived. Six fetuses with large lung masses and persistent mediastinal compression near term underwent ex-utero intrapartum therapy-to-resection procedures, and 3 fetuses with hydrops underwent serial thoracentesis. Congenital cystic adenomatoid malformation volume ratio correlated strongly with the development of hydrops and the need for fetal therapy with an area under the receiver operating characteristic curve of 0.96 (P < .0001) and 0.88 (P < .0001), respectively. Of 18 fetuses with a CVR greater than 2.0 compared with 2 (3%) of 60 with a CVR of 2.0 or less, 10 (56%) required fetal intervention (P < .0001).

Conclusion

Congenital cystic adenomatoid malformation volume ratio correlates strongly with the development of fetal hydrops and the need for fetal intervention. A threshold value of 2.0 yields the most powerful statistical results.     

Large fetal congenital cystic adenomatoid malformations: growth trends and patient survival

Purpose

The prognosis for fetuses with large congenital cystic adenomatoid malformations (CCAMs) remains uncertain. This study examined the natural history of large fetal CCAMs managed expectantly at a major referral center.

Methods

A 5-year retrospective review was conducted on fetuses diagnosed with a thoracic lesion (n = 59). Large CCAMs were identified on prenatal imaging and followed longitudinally. Perinatal outcomes were assessed.

Results

Twelve (20.3%) fetuses had large CCAMs in the absence of other congenital anomalies. Peak CCAM size occurred at 25.3 ± 3.6 weeks' gestation. Serial magnetic resonance volumetry demonstrated a trend toward decreasing CCAM mass volume relative to thoracic cavity volume over time. Overall, 6 patients, including 3 with signs of early hydrops, showed a marked regression of their lesions relative to estimated fetal weight. Five fetuses required an emergent intervention postnatally, including extracorporeal membrane oxygenation support (n = 1), cyst aspiration (n = 1), and lung resection (n = 5). Overall survival was 75%, with severe hydrops before 30 weeks seen in all 3 deaths.

Conclusion

Large fetal CCAMs tend to peak in size at 25 weeks' gestation and are characterized by in utero diminution relative to overall fetal growth. The prognosis for most fetuses with large CCAMs remains quite favorable under careful perinatal management.     

Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome

Purpose

To understand the natural history and define indications for fetal intervention in sacrococcygeal teratoma (SCT), the authors reviewed all cases of fetal SCT presenting for evaluation.

Methods

Prenatal diagnostic studies including ultrasound scan, magnetic resonance imaging (MRI), echocardiography and pre- and postnatal outcomes were reviewed in 30 cases of SCT that presented between September 1995 and January 2003.

Results

The mean gestational age (GA) at presentation was 23.9 weeks (range, 19 to 38.5) with 3 sets of twins (10%). Overall outcomes included 4 terminations, 5 fetal demises, 7 neonatal deaths, and 14 survivors. Significant obstetric complications occurred in 81% of the 26 continuing pregnancies: polyhydramnios (n = 7), oligohydramnios (n = 4), preterm labor (n = 13), preeclampsia (n = 4), gestational diabetes (n = 1), HELLP syndrome (n = 1), and hyperemesis (n = 1). Fetal intervention included cyst aspiration (n = 6), amnioreduction (n = 3), amnioinfusion (n = 1), and open fetal surgical resection (n = 4). Indications for cyst aspiration and amnioreduction were maternal discomfort, preterm labor, and prevention of tumor rupture at delivery. Although 15 SCTs were solid causing risk for cardiac failure, only 4 fetuses met criteria for fetal debulking based on ultrasonographic and echocardiographic evidence of impending high output failure and favorable anatomy at 21, 23.6, 25, and 26 weeks’ gestation. Intraoperative events included maternal blood transfusion (n = 1), fetal blood transfusion (n = 2), chorioamniotic membrane separation (n = 2), and fetal arrest requiring successful cardiopulmonary resuscitation (CPR) (n = 1). In the fetal resection group, 3 of 4 survived with mean GA at delivery of 29 weeks (range, 27.6 to 31.7 weeks), mean birth weight of 1.3 kg, hospital stay ranging from 16 to 34 weeks, and follow-up ranging from 20 months to 6 years. Postnatal complications in the fetal surgery group included neonatal death (n = 1, secondary to premature closure of ductus arteriosus with cardiac failure), embolic event (n = 1, resulting in unilateral renal agenesis, jejunal atresia), chronic lung disease (n = 1), and tumor recurrence (n = 1).

Conclusions

For fetal SCT, the rapidity at which cardiac compromise can develop and the high incidence of obstetric complications warrant close prenatal surveillance. Amnioreduction, cyst aspiration, and surgical debulking are potentially life-saving interventions.     

Is a vesicoamniotic shunt intrinsically bad? Shunting a normal fetal bladder

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.     

Lentiviral vector-mediated, in vivo gene transfer to the tracheobronchial tree in fetal rabbits

Background/Purpose

Fetal gene replacement is a novel, potential therapy for antenatally diagnosed monogenic disorders. The purpose of this study was to evaluate in vivo techniques of lentiviral (LV) vector-mediated gene transfer to the tracheobronchial tree in a rabbit model of fetal gene therapy.

Methods

Via triple plasmid transfection, vesicular stomatitis virus-G-pseudotyped LV vector containing green fluorescent protein (GFP) reporter gene under the control of a cytomegalovirus promoter was constructed.In vivo gene transfer of 5 × 10⁶ LV particles to fetuses of time-mated NZW rabbits (term = 31 days) was attempted using 2 techniques: (1) direct amniotic injection (gestation = 24-26 days) and (2) direct tracheal injection (gestation = 26 days). Injected fetuses and saline-injected littermate controls were delivered and killed on gestational day 30. Fetal and maternal tissues were analyzed.

Results

Both in vivo techniques produced gene transfer to fetal tissues (trachea, lung, liver, intestine), including those of some controls. In one prep, GFP DNA was identified in maternal lung.

Conclusions

Lentiviral vector-mediated GFP gene transfer to fetal rabbit tracheobronchial epithelium occurs within 4 days of transfection by both amniotic injection or direct fetal tracheal injection. This in vivo model confirms bioavailability of vector through amniotic fluid with some cross-infection of adjacent fetuses. Vector access to fetal tissues appears to be by both luminal and hematogenous routes. Transplacental gene transfer from fetus to mother may occur in this model.     

Evolution of impaired renal function after external continent urinary diversion (Mitrofanoff principle)

Objective

To study the evolution of impaired renal function after external continent urinary diversion (Mitrofanoff principle) (ECUD-M) associated with ileocystoplasty.

Patients and Methods

Over 18 years from 1992 to 2009, ECUD-M with ileocystoplasty was performed in 120 patients with mean age of 25.5 years. Renal impairment was evident in 43 patients (17 children and 26 adults).

Results

Ninety percent of patients demonstrated a neurologic bladder and mild to moderate renal failure. Initially, all patients underwent continuous bladder drainage for a mean of 3 weeks. Renal function improved in 35 patients, although with persistent mild renal insufficiency. The other patients demonstrated moderate persistent residual renal insufficiency. During a mean follow-up of 10 years (range, 1-18 years), renal function returned to normal in 13 patients, stabilized at lower values in 15, and remained moderate in 5. After a mean follow-up of 8 years (range, 6-12 years), renal failure gradually worsened, increasing to higher values in 6 patients and leading to hemodialysis in 4. One patient underwent living-donor kidney transplantation, with good evolution.

Conclusion

ECUD-M with ileocystoplasty can lead to normalization unless stabilizationof impaired residual renal function by eliminating the obstructive factor provides self-adequate management of the diversion. The procedure delays for the need forhemodialysis therapy, and enables patients to prepare for kidney transplantation into apreviously reconstructed lower urinary tract.     

Administration of prenatal betamethasone suppresses the adrenal-hypophyseal axis in newborns with congenital diaphragmatic hernia

Background

Congenital diaphragmatic hernia (CDH) is a condition that is characterized by pulmonary hypoplasia and pulmonary hypertension. Prenatal betamethasone often is administered to fetuses with CDH to improve pulmonary function. In this study, the authors investigate the possible role of the adrenal-hypophyseal axis in CDH in an animal model and subsequently in human infants with CDH.

Methods

Twin fetal sheep underwent creation of DH or a sham thoracotomy, and levels of plasma and lung ACTH and plasma cortisol were compared. For the human studies, plasma levels of ACTH, cortisol, and DHEA were measured in cord blood samples collected from 9 CDH (5 that received prenatal betamethasone) and compared with those of 14 normal newborns. In both studies, ACTH and cortisol levels were determined by radioimmunoassay (RIA). Human (DHEA) levels were determined by ELISA.

Results

Plasma ACTH and cortisol levels were elevated in fetal DH sheep compared with sham-operated controls; however, levels of ACTH in lung tissues were not different. Human newborns with CDH who have been exposed to prenatal steroids have significantly lower plasma ACTH, cortisol, and DHEA levels than normal newborns and CDH newborns not exposed to prenatal betamethasone.

Conclusions

In an ovine model of CDH, the adrenal-hypophyseal axis appears up-regulated in DH fetuses compared with sham-operated animals. Conversely, the adrenal-hypophyseal axis in human CDH newborns appears normal but is suppressed by the administration of prenatal betamethasone.     

In utero repair of gastroschisis in the sheep (Ovis aries) model

Objective

Gastroschisis is associated with inflammatory changes in the exposed bowel which leads to intestinal dysmotility after postnatal repair. The insult is a combined effect of amniotic fluid exposure and mechanical constriction. We hypothesized that in utero anatomic repair is possible in a sheep model, and that it may halt the inflammatory damage caused by both mechanisms.

Methods

Gastroschisis was surgically created in mid-gestation (day 75) in 8 sheep fetuses. On gestational day 100, 2 fetuses underwent open fetal gastroschisis repair, where the eviscerated bowel was returned to the peritoneal cavity, and the abdominal wall was primarily closed. All fetuses were harvested at 135 days of gestation.

Results

Six fetuses survived the initial operation, and both fetuses that underwent gastroschisis repair survived to term. At 100 and 135 days of gestation, the eviscerated bowel showed progressive signs of inflammation and peel development. The gross and microscopic inflammatory changes in the gastroschisis bowel at 100 days of gestation were completely resolved at term following in utero repair.

Conclusion

In utero anatomic repair of gastroschisis is possible in mid-gestation in the fetal lamb model, and it appears to ameliorate the inflammatory process.     

Temporary percutaneous and permanent gastric electrical stimulation in children younger than 3 years with chronic vomiting

Background

The aim was to investigate whether young children with drug-refractory nausea and vomiting can be treated with gastric electrical stimulation (GES) in a similar way as adults and to evaluate whether temporary percutaneous gastric electrical stimulation (TPGES) can be used in the pediatric population to select the patients who are responders to GES treatment. We report the clinical results in 3 children between 2 and 3 years of age. To the best of our knowledge, these are the youngest patients treated with GES.

Methods

Three patients younger than 3 years with intractable vomiting underwent TPGES. Custom-made leads were percutaneously implanted in the gastric wall under gastroscopic guidance. Symptoms were recorded daily during the TPGES stimulation time (12-40 days). Responders were offered permanent GES treatment.

Results

There were no technical problems. All 3 patients were responders to TPGES. They are now treated with surgically implanted permanent GES and reported greater than 50% vomiting reduction at last visit.

Conclusion

Children younger than 3 years can be treated with GES in a similar way as adolescents and adults. Temporary percutaneous GES is a safe, feasible technique even in small children, with the possibility to perform the test over several weeks to select responders to GES treatment.     

High-risk fetal congenital pulmonary airway malformations have a variable response to steroids

Background/Purpose

Anecdotal reports suggest that maternal steroids may arrest the growth of congenital pulmonary airway malformations (CPAMs), preventing or reversing hydrops. We reviewed our experience with CPAMs to determine the fetal response to steroid therapy.

Methods

This study is a retrospective review of all fetal CPAMs from 2004 to 2008. Fetuses with high-risk CPAMs that received at least one course of steroids were identified. Fetal magnetic resonance imaging and ultrasound data were used to classify the CPAMs, identify hydrops fetalis and follow the fetuses poststeroid dosing.

Results

Forty-four fetuses with CPAM were identified. Fifteen patients were found to have received at least one course of steroids. Thirteen were hydropic and 2 were nonhydropic. Seven of the 13 hydropic fetuses (54%) showed an initial response to steroid administration, whereas the 2 nonhydropic high-risk fetuses progressed to birth without developing hydrops. Seven of the 15 patients, however, resulted in fetal demise or early postnatal death, giving a survival rate of 53%.

Conclusions

High-risk CPAMs have a variable response to steroids. This variable response demonstrates the need for a placebo-controlled randomized study to more accurately determine the effect of steroids on hydrops and CPAM growth rates. Repeated steroid courses may not be helpful, and progression in CPAM volume to head circumference ratio (CVR) or hydrops should prompt open fetal surgery to prevent irreversible fetal insult.     

Management of fetal mediastinal teratoma

Background/Purpose

Mediastinal teratomas are rare congenital germ cell tumors that prenatally can compress mediastinal structures and cause hydrops. Two possible presentations of massive fetal mediastinal teratoma include hydrops leading to fetal demise, or fetal esophageal and airway compression causing late-gestation polyhydramnios and preterm labor. The authors present 2 cases of fetal mediastinal teratoma that illustrate successful strategies for either of these presentations.

Methods

A 37-year-old woman carrying a fetus with a mediastinal mass and secondary hydrops at 23 weeks of gestation underwent in utero resection of the mass. Delivery was by cesarean delivery at 25 weeks because of preterm labor. A 24-year-old mother carrying a fetus with a mediastinal mass and severe polyhydramnios at 36 weeks of gestation underwent an ex utero intrapartum therapy procedure for establishment of an airway and tumor resection on uteroplacental support.

Results

These strategies resulted in physiologic improvement in the first case and controlled resection and resuscitation in the second. The first patient had significant sequelae of prematurity including bronchopulmonary dysplasia but is currently well at 9 months of age. The second patient is well at 1 year of age.

Conclusions

Massive fetal mediastinal teratoma can result in fetal or neonatal mortality by a variety of mechanisms. Optimal prenatal and perinatal management is required to salvage fetuses compromised by this lesion.     

Fetal gastrointestinal motility in a rabbit model of gastroschisis

Purpose

Gastrointestinal (GI) dysmotility occurs frequently in full-term infants with gastroschisis (GS). Although controversial, preterm delivery of infants with GS has been advocated to prevent the development of GI dysmotility, and understanding the etiology of gestational-related bowel injury may lead to prenatal therapies. Using a fetal rabbit model, the authors assessed in vivo and in vitro GI motility in preterm GS fetuses.

Methods

On gestation day 24 (term, 31), surgery was performed in maternal rabbits and GS induced in fetuses, whereas control fetuses underwent sham procedures. On gestation day 29, both groups of fetuses received ultrasound-guided intragastric injection of fluorescein and colored microspheres. Two hours after injection, fetuses were delivered by cesarean section and stomach and small intestine harvested intact. “GI motility” was calculated as the distance traveled by fluorescein divided by total length. In vitro studies of fetal gastric muscle strips contractility responses to bethanechol, a cholinergic agonist, were assessed in an organ bath system. Data were analyzed as paired and unpaired t tests and expressed as means ± SEM.

Results

GS reduced fetal body weight and intestinal length compared with controls (28.4 ± 1.4 v. 33.5 ± 1.5 g, 36.9 ± 1.8 v. 25.9 ± 1.2 cm; P < .05, respectively). Fetuses with GS showed markedly reduced in vivo GI motility (51.4 ± 2.9 v 24.8 ± 2.7%; P < .05) and in vitro gastric contractile tension (769 ± 53 v 396 ± 26 mNcm²; P < .05).

Conclusions

GI exposure to amniotic fluid reduces intestinal motility and gastric contractility functions in the preterm rabbit fetus. The results suggest that GS-associated impairment of GI neuromuscular functions occurs in utero, before term, and may be responsive to manipulation of amniotic fluid content or other therapeutic interventions.     

Evaluation of an antireflux procedure for colonic interposition in pediatric esophageal replacements

Background

In 1989, we introduced a 1-stage procedure with orthotopic colonic transplants for esophageal stenosis. A pitfall of this procedure is frequent reflux and/or stasis in the transplants from the cologastric anastomosis. Since 1993, we have used a new antireflux wrap (ARW) using an anterior wrap technique similar to the Dor procedure but fixed to the right crus of the diaphragm.

Purpose

The purpose of the study was to evaluate ARWs.

Method

From 1993 to 2008, the records of 67 patients with an ARW were compared with 27 without ARW (either operated on before 1993 or ARW was not appropriate) after colonic transplant for caustic esophageal stenosis. Both groups otherwise underwent the same surgical procedure. Postoperative esophagograms done on postoperative day 10 were reviewed for the presence of gastrocolonic reflux and stasis in the transplant.

Results

The reflux rate on the initial esophagogram was reduced from 48.1% to 7.5% using ARW. The incidence of reflux on later esophagograms was 40.0% with no ARW and 21.4% with ARW. The 25% long-term rate of stasis in the colonic transplant was not increased with ARW.

Conclusions

A loose ARW in patients with colonic esophageal replacements reduces gastrocolic reflux without increasing the rate of stasis. In the long term, children adapt better to stasis than to reflux and are thus protected from occult inflammation.     

Morphologic evaluation of regenerated small bowel by small intestinal submucosa

Background/Purpose

Previous studies have shown small intestinal submucosa (SIS) can be used as biodegradable scaffolds in tissue engineering small intestine. The purpose of this study is to evaluate the regeneration of neointestine and its morphology using SIS.

Methods

A 2-cm tubular SIS graft from Sprague Dawley rat donors was interposed in the middle of a 6-cm ileal Thiry-Vella loop of Lewis rats, which was used to construct an ileostomy. The grafts were harvested at each of the time points ranging from 2 weeks to half a year after implantation, and native small intestine and grafts were investigated for morphology using histology and immunohistochemistry.

Results

At the early postoperative period, SIS grafts were colonized by numerous inflammatory cells. A mucosal epithelial layer began to line the luminal surface of the graft by 4 weeks, and by 12 weeks, the luminal surface was covered completely by a layer of neomucosa. Neomucosa with typical small bowel morphology was characterized by a columnar epithelial cell layer with goblet cells, Paneth cells, absorptive enterocytes, and enteroendocrine cells. Significant differences between neomucosa by 12 weeks and 24 weeks in the measurements of mucosal thickness, villus height, and crypt depth were found. The outer walls of SIS grafts were composed of distinct bundles of well-formed smooth muscle-like cells with some fibrovascular tissue.

Conclusions

This initial study suggests that tissue engineering neointestine using SIS can develop structural features of the normal intestine. Small intestinal submucosa might be a viable material in the creation of neointestine for patients suffering short bowel syndrome.     

Anastomotic leakage after laparoscopic resection of rectal cancer: The impact of fibrin glue

Background

The aim of this study was to evaluate whether the use of fibrin glue as a sealant over an anastomosis is a risk factor for anastomotic leakage after laparoscopic rectal cancer surgery.

Methods

Prospective data were collected from 223 patients with rectal cancer who underwent laparoscopic resection without defunctioning stoma.

Results

A total of 104 patients underwent laparoscopic rectal resection, followed by the application of fibrin glue over the stapled anastomosis, while 119 underwent surgery alone. No difference in clinically significant leakage was observed between the fibrin and the nonfibrin groups (5.8% vs 10.9%, P = .169). In multivariate analysis, extraperitoneal tumor location and operation duration >220 minutes were independently associated with anastomotic leakage.

Conclusions

Significant predictors of anastomotic leakage include extraperitoneal tumor location and operation length >220 minutes. Fibrin glue application over the stapled anastomosis was not found to be significantly associated with anastomotic leakage.     

Congenital high airway obstruction syndrome: natural history and management

Background

Congenital high airway obstruction syndrome (CHAOS) is a life-threatening condition with a poorly understood natural history.

Methods

A retrospective review of five patients with CHAOS between 1997 and 2002 was performed.

Results

All fetuses had large echogenic lungs, dilated airways, inverted diaphragms, and massive ascites. One fetus with a laryngeal cyst was terminated at 22 weeks. A twin fetus with findings suggestive of a tracheal web had progressive hydrops, which led to fetal demise. The remaining 3 patients delivered via the ex utero intrapartum treatment (EXIT) procedure survived. The first patient tolerated progressive hydrops for 12 weeks in utero. He had tracheal atresia but underwent laryngotracheoplasty successfully. He is the first long-term CHAOS survivor and is speaking at 5 years of age. The 2 patients with relatively stable lung volumes prenatally have laryngeal atresia with a pinpoint posterior laryngeal fistula. Their postnatal clinical courses were much more benign than the first survivor.

Conclusions

The prenatal natural history and postnatal course of CHAOS depends on whether the airway obstruction is complete. The EXIT procedure offers the potential for salvage of this otherwise lethal condition. Hydrops may be well tolerated prenatally for weeks with potential resolution if airway fistulization is present.     

The early effects of urinary tract obstruction on glomerulogenesis

Purpose

Our obstructive uropathy model in fetal lambs showed that renal cystic changes appeared 3 weeks after obstruction. In this study, the authors investigated the changes resulting from complete urinary tract obstruction in the first 7 days after obstruction.

Methods

An obstructive uropathy was created in fetal lambs at 60 days’ gestation by ligating the urethra and urachus. They were delivered 48 hours, 3 days, 5 days, and 7 days later by cesarian section. The kidneys were removed and processed for histologic examination.

Results

Eighteen fetuses were operated on and 15 (4 at 48 hours, 4 at 3 days, 2 at 5 days, and 5 at 7 days; 83%) survived. Macroscopically, bladder dilatation and slightly dilated ureters were identified from 48 hours. Microscopically, dilatation of proximal tubules started from 48 hours after obstruction and increased by 7 days. Glomerular cysts in the nephrogenic zone also were identified from 48 hours. Dysplastic changes were not found.

Conclusions

The first areas in the developing kidney that suffer damage after obstructive uropathy are the proximal tubule and the nephrogenic zone. This change started 48 hours after obstruction. Shunting procedures need to be performed considerably earlier than previously thought.     

Vesicoamniotic shunt for complete urinary tract obstruction is partially effective

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.