Prenatal Decompression Research Articles

Isolated Fetal Ventriculomegaly: Diagnosis and Treatment in the Prenatal Period.

Fetal ventriculomegaly (VM) is a defect of the central nervous system, typically diagnosed during the second-trimester ultrasound in fetuses with an atrial diameter (AD) of >10 mm. Non-isolated ventriculomegaly (NIVM) is heterogeneous in nature, coexisting with additional intracranial and/or extracranial malformations and genetic syndromes, resulting in an unfavorable prognosis for the further development of the child. Both the pregnancy management and counseling are dependent on the findings of combined ultrasound/MRI, genetic testing, and gestational age at diagnosis. The purpose of this review is to propose a hypothesis that diagnostic advancements allow to define the process of identification of the isolated forms of VM (IVM). Based on the evidence presented in the literature, we consider whether prenatal decompression for severe isolated VM (ISVM) is supported by the experimental trials and whether it might be implemented in clinical practice. Also, we describe the evolution of the diagnostic methods and expert opinions about the previously used prenatal decompression techniques for ISVM. In conclusion, we introduce the idea that fetal surgery centers have either reached or nearly reached the necessary level of expertise to perform such procedures. Endoscopic cystoventriculostomy (ETV) appears to be the most promising, as it is associated with minimal perinatal complications and favorable neurological outcomes in the neonatal period. Randomized trials with long-term neurodevelopmental follow-up of children who underwent prenatal decompression due to ISVM are necessary.

Prenatal extravasation of urine seems to preserve renal function in boys with posterior urethral valves

A posterior urethral valve(PUV) may lead to extravasation of urine, resulting in prenatal ascites and/or perirenal urinoma. Extravasation has been presumed to act as a pop-off mechanism, preserving renal function, but previous reports addressing this presumption have been inconclusive. The present study compares renal function in patients with PUV with and without extravasation. Sixty boys with a confirmed diagnosis of PUV as neonates (gestational age [GA]<44 weeks) throughout 2001-2016 were included. Clinical data were collected from medical records. Renal function was assessed by nadir plasma creatinine, creatinine at the last follow-up, and glomerular filtration rate (GFR) at the last follow-up. The GFR was estimated using the Schwartz formula. Renal function was classified according to the kidney disease: improving global outcomes (KDIGO) guidelines' grades of chronic kidney disease (CKD). Glomerular filtration rate > 90ml/min/1.73m2 at the last follow-up was classified as normal renal function. Twelve patients (20%) had ascites and/or urinoma, and 48 (80%) did not. GA and birth weight were not different in patients with and without extravasation. PUV was suspected from prenatal ultrasound findings in 66.7% of the patients in both groups. Median nadir creatinine was 21 (range, 11-33) μmol/L in boys with ascites/urinoma, and all values were within the age-adjusted reference values. Nadir creatinine was 23 (14-199) μmol/L in boys without extravasation, and it was above the normal range in 14 boys. The incidence of elevated nadir creatinine was significantly different in the two groups (p<0.025). One of the 12 patients with extravasation developed chronic renal failure (CKD 3). In the group of 48 patients without extravasation, 20 (42%) had chronic renal failure grade 2-5, and among these, 5 patients have had a renal transplant (CKD grade 5). The prevalence of CKD grade 2-5 was statistically different in the two groups (p=0.03). These findings are presented in the summary figure. Extravasation of urine was found in 12 of 60 (20%) boys with PUV. These patients had significantly lower prevalence of CKD at the last follow-up than patients without extravasation. This finding is important in prenatal counseling. It also indicates that prenatal decompression of the bladder and upper tract is beneficial in patients with PUV, which is relevant to the discussion of prenatal intervention in these fetuses.

Renal tract malformations: perspectives for nephrologists

Renal tract malformations are congenital anomalies of the kidneys and/or lower urinary tract. One challenging feature of these conditions is that they can present not only prenatally but also in childhood or adulthood. The most severe types of malformations, such as bilateral renal agenesis or dysplasia, although rare, lead to renal failure. With advances in dialysis and transplantation for young children, it is now possible to prevent the early death of at least some individuals with severe malformations. Other renal tract malformations, such as congenital pelviureteric junction obstruction and primary vesicoureteric reflux, are relatively common. Renal tract malformations are, collectively, the major cause of childhood end-stage renal disease. Their contribution to the number of adults on renal replacement therapy is less clear and has possibly been underestimated. Renal tract malformations can be familial, and specific mutations of genes involved in renal tract development can sometimes be found in affected individuals. These features provide information about the causes of malformations but also raise questions about whether to screen relatives. Whether prenatal decompression of obstructed renal tracts, or postnatal initiation of therapies such as prophylactic antibiotics or angiotensin blockade, improve long-term renal outcomes remains unclear.

Hypoplastic Left Heart Syndrome With Intact or Highly Restrictive Atrial Septum: Surgical Experience From a Single Center

The presence of an intact or highly restrictive atrial septum (I/HRAS) has long been recognized as a predictor of poor outcome among patients with hypoplastic left heart syndrome (HLHS), although the rarity of this condition has precluded conclusive study. The purpose of this review is to summarize recent surgical outcomes for these patients at our center and to identify predictors. We retrospectively identified all neonates with a diagnosis of HLHS and I/HRAS who underwent stage I palliation at Children's Hospital Boston between January 2001 and December 2006. Chart review enabled analysis of patient and procedural variables. All 32 patients underwent left atrial decompression in utero or postnatally before surgery. Fourteen patients (44%) underwent fetal intervention, either atrial septoplasty (n = 9) or aortic valvuloplasty (n = 5). Twenty-nine of the 32 patients had postnatal left atrial hypertension and underwent transcatheter atrial septoplasty as neonates before surgery; 3 did not require postnatal atrial septoplasty after successful fetal atrial septoplasty. After stage I, hospital survival was 69% (22 of 32). Need for shunt revision (p = 0.02) and for extracorporeal membrane oxygenation use (p < 0.001) were associated with hospital mortality. Survival at 6 months was 69% for patients who had fetal intervention, and 38% for those who were treated only postnatally (p = 0.2). Surgical outcome for patients with HLHS and I/HRAS continues to improve. Prenatal decompression of the left atrium may be associated with greater hospital survival. Proposed effects of fetal intervention on lung pathology and longer-term survival are subjects for future study in this unique group of patients.

Prenatal Diagnosis of a Ranula With 2- and 3-Dimensional Sonography and Sonographically Guided Aspiration

Journal of Ultrasound in MedicineVolume 25, Issue 11 p. 1499-1502 Case Report Prenatal Diagnosis of a Ranula With 2- and 3-Dimensional Sonography and Sonographically Guided Aspiration Pedro Pires MD, PhD, Pedro Pires MD, PhD Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Search for more papers by this authorMayra Pereira MD, PhD, Mayra Pereira MD, PhD Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Search for more papers by this authorLuiz Machado MD, PhD, Luiz Machado MD, PhD Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Search for more papers by this authorFernando Bonilla-Musoles MD, PhD, Corresponding Author Fernando Bonilla-Musoles MD, PhD cegiob@interbook.net Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Address correspondence to Fernando Bonilla-Musoles, MD, PhD, Navarro Reverter 11, 46004 Valencia, Spain.Search for more papers by this author Pedro Pires MD, PhD, Pedro Pires MD, PhD Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Search for more papers by this authorMayra Pereira MD, PhD, Mayra Pereira MD, PhD Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Search for more papers by this authorLuiz Machado MD, PhD, Luiz Machado MD, PhD Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Search for more papers by this authorFernando Bonilla-Musoles MD, PhD, Corresponding Author Fernando Bonilla-Musoles MD, PhD cegiob@interbook.net Serviço de Medicina Fetal, Centro Integrado de Saúde Amaury de Medeiros/Universidade de Pernambuco, Recife, Brazil.Address correspondence to Fernando Bonilla-Musoles, MD, PhD, Navarro Reverter 11, 46004 Valencia, Spain.Search for more papers by this author First published: 01 November 2006 https://doi.org/10.7863/jum.2006.25.11.1499Citations: 10Read the full textAboutPDF ToolsRequest permissionExport citationAdd to favoritesTrack citation ShareShare Give accessShare full text accessShare full-text accessPlease review our Terms and Conditions of Use and check box below to share full-text version of article.I have read and accept the Wiley Online Library Terms and Conditions of UseShareable LinkUse the link below to share a full-text version of this article with your friends and colleagues. Learn more.Copy URL Share a linkShare onFacebookTwitterLinked InRedditWechat Citing Literature Volume25, Issue11November 2006Pages 1499-1502 RelatedInformation

The management of fetal ovarian cysts

Background/Purpose: Ovarian torsion causing the loss of an ovary represents the most common complication of fetal ovarian cysts and occurs more frequently before than after birth. Thus, treatment of fetal simple ovarian cysts should be performed antenatally; however, criteria for prenatal decompression still need to be evaluated. Previous experience of the authors showed that large simple cysts have a poor outcome, whereas preliminary attempts of their “in utero” aspiration were all successful and uneventful. The authors evaluated the outcome of fetal simple ovarian cysts after prenatal aspiration and considered criteria for this procedure. The outcome of cysts showing a prenatal ultrasound pattern of torsion also was studied. Methods: This prospective study includes 73 ovarian cysts (48 simple, 25 showing torsion) diagnosed in 72 fetuses from June 1992 to June 1999, and followed up until spontaneous resolution or surgery. Prenatal aspiration was performed in the case of simple cysts ≥5 cm in diameter. The outcome of these cysts was compared with that of similar cysts not aspirated in the authors' previous study (X2).10 Cysts with an US pattern of torsion persisting at birth were operated on. The outcome of simple cysts less than 5 cm and cysts with a prenatal ultrasound appearance of torsion also was evaluated. Results: Prenatal decompression was performed without any complications in 14 cases: 12 (86%; 95% CI: 0.68 to 1.00) regressed subsequently; 2 (14%; 95% CI: 0.00 to 0.32) showed torsion postnatally. This outcome is significantly better than that of similar cysts not aspirated in the authors' previous study10 (P =.0002). Among the 34 simple cysts less than 5 cm, 26 (76%; 95% CI: 0.62 to 0.90) resolved spontaneously; 8 (24%; 95% CI: 0.10 to 0.38) had complications, 7 of which showing torsion (diameter at evidence of torsion, 4.4 cm [median]; range, 3.3 to 5.2 cm). Among the 34 cysts showing torsion (25 with initial US pattern of torsion + 9 subsequently complicated simple cysts), 24 (71%; 95% CI: 0.56 to 0.86) required oophorectomy; 9 (26%; 95% CI: 0.11 to 0.41) spontaneously disappeared at ultrasound, one of which required surgery for intestinal obstruction secondary to adhesion of a necrotic ovary; one patient (3%; 95% CI: 0.00 to 0.09) was lost to follow-up. Conclusions: Prenatal aspiration of ovarian cysts appears effective and safe: a “cutoff” of 4 cm should be investigated. Cysts with ultrasound pattern of torsion persisting postnatally require surgery; options for their management, when sonographically disappearing and asymptomatic, need to be investigated. J Pediatr Surg 37:25-30. Copyright © 2002 by W.B. Saunders Company.

URETHRAL ATRESIA: LONG-TERM OUTCOME IN 6 CHILDREN WHO SURVIVED THE NEONATAL PERIOD

Urethral atresia is incompatible with life unless an alternative communication between the bladder and amniotic sac exists. Although antenatal intervention may improve perinatal mortality, clinical consequences remain. We outline the outcome after treatment of 6 patients born with urethral atresia. We reviewed the charts of 6 patients with urethral atresia treated at the Children's Hospital of Michigan between 1982 and 1999. Patient age ranged from 3 to 17 years (mean 9). All patients were males (46 XY) and presented at birth or in early infancy. Oligohydramnios was demonstrated in 4 of the 6 patients (67%). A vesico-amniotic shunt had been placed in 2 patients (at more than 30 weeks of gestation and in 1 at 17 weeks of gestation). The remaining 3 patients presented at birth with either a vesicocutaneous fistula or patent urachus. Bilateral or unilateral hydronephrosis was identified in 4 patients while 2 had severe renal dysplasia. Cystography identified moderate to high grade vesicoureteral reflux in all patients, and 5 (83%) had the prune belly syndrome. Mean serum creatinine at age 1 year was 1.3 mg/dl (range 0.5 to 2.1). Renal failure occurred in 5 patients (83%) before age 10 years and 4 of them have received a renal transplant. An average of 7.8 (range 9 to 14) urological procedures were performed on each patient. Progressive urethral dilation was not successful in the majority of our cases and ultimately 67% required some form of supravesical diversion. Our study demonstrates that urethral atresia is not necessarily fatal. Prenatal decompression allows survival and in some cases may even lead to normal bladder and renal function. A complicated clinical course requiring extensive reconstruction is to be expected.

Urethral atresia: long-term outcome in 6 children who survived the neonatal period.

Urethral atresia is incompatible with life unless an alternative communication between the bladder and amniotic sac exists. Although antenatal intervention may improve perinatal mortality, clinical consequences remain. We outline the outcome after treatment of 6 patients born with urethral atresia. We reviewed the charts of 6 patients with urethral atresia treated at the Children's Hospital of Michigan between 1982 and 1999. Patient age ranged from 3 to 17 years (mean 9). All patients were males (46 XY) and presented at birth or in early infancy. Oligohydramnios was demonstrated in 4 of the 6 patients (67%). A vesico-amniotic shunt had been placed in 2 patients (at more than 30 weeks of gestation and in 1 at 17 weeks of gestation). The remaining 3 patients presented at birth with either a vesicocutaneous fistula or patent urachus. Bilateral or unilateral hydronephrosis was identified in 4 patients while 2 had severe renal dysplasia. Cystography identified moderate to high grade vesicoureteral reflux in all patients, and 5 (83%) had the prune belly syndrome. Mean serum creatinine at age 1 year was 1.3 mg/dl (range 0.5 to 2.1). Renal failure occurred in 5 patients (83%) before age 10 years and 4 of them have received a renal transplant. An average of 7.8 (range 9 to 14) urological procedures were performed on each patient. Progressive urethral dilation was not successful in the majority of our cases and ultimately 67% required some form of supravesical diversion. Our study demonstrates that urethral atresia is not necessarily fatal. Prenatal decompression allows survival and in some cases may even lead to normal bladder and renal function. A complicated clinical course requiring extensive reconstruction is to be expected.

Histology of the fetal prune belly syndrome with reference to the efficacy of prenatal decompression.

Deficient abdominal musculature, complex abnormalities of urinary tracts and bilateral abdominal cryptorchidism represent the basic characteristics of prune belly syndrome (PBS). Although prenatal diagnosis of PBS is rarely made, because of the wide variety of ultrasonographic images, reported cases have gradually increased. Once a fetus suspected of having PBS is found, it is sometimes difficult for the pediatric urologists to decide how to treat them. The histology of the kidney and urinary tracts in fetuses with PBS was reviewed in order to give suggestions on the management of prenatal cases. Autopsy records of nine fetuses (5 males, 2 females and 2 undetermined) with characteristically distended and deficient abdominal wall were reviewed. Gestational age (GA) at detection ranged from 12 to 25 weeks and at delivery from 13 to 32 weeks. Renal histology in two fetuses showed earlier than normal disappearance of cortical nephrogenic zone replaced by cortical cysts and dysplastic structures. The nephrogenic zone was retained in five fetuses which were younger than GA 20 weeks. While the number of glomeruli along the medullary ray was normal for the age in three fetuses younger than GA 20 weeks, it was decreased in all others. Bladder histology was variable showing both increased musculature and defective or dysplastic muscles. There was a tendency for connective tissues in the bladder wall to increase in proportion to GA, The ureter revealed scarcity of muscle bundles among dense connective tissue. The urethra was atretic in eight fetuses. The clinical implication from the renal histology is that decompression of the urinary tract should be done before GA 20 weeks. However, the early fetal treatment appears to have no effect on the urodynamics in this disorder with deficient musculature.

Histological study of fetal hydronephrosis. Development background of prenatal treatment

It is still unclear whether prenatal decompression of the dilated urinary tracts can preserve renal development and function. Following the previous report on the relationship between the nephrogenesis and the gestational weeks in normal fetuses, we studied the hydronephrotic kidneys to find the histological background of the fetal treatment. Of the fetuses autopsied in the Department of Pathology of our Institute from 1982 to 1992, 32 kidneys from 21 fetuses associated with dilatation of the upper urinary tracts irrespective of the presence of urethral obstruction on macroscopic inspections were reviewed. The underlying disorders causing hydronephrosis included urethral obstruction in 7, vesico-intestinal fissure in 3, hydrometrocolpos in 2 and double ureter in 1. The etiology of the hydronephrosis was not clarified in another 8. The position and configuration of the ureteral orifices were not described in the autopsy reports. Microscopic examination was done to define the radial glomerular count (RGC), presence or absence of the nephrogenic zone, cortical cysts and dysplastic elements, changes of the collecting tubules and interstitium. Of the kidneys 19 had cortical cysts of various sizes. In some kidneys, the normal cortex was mixed with pathological segments where there were subcapsular small cysts and tubular dilatation. Nephrogenic zone, which was observed in specimens of 33-weeks fetus with moderate hydronephrosis, was already missing by 28 weeks in severe hydronephrosis. Collecting tubules were dilated in 12 kidneys with mild to moderate hydronephrosis. Tubular atrophy and increase in the interstitium were seen in 12 kidneys, eleven of which were of the fetus over 30 weeks.(ABSTRACT TRUNCATED AT 250 WORDS)

Fetal intervention in obstructive uropathy: Prognosticindicators and efficacy of intervention

Management of the fetus with bilateral hydronephrosis is controversial; ability to predict outcome and efficacy of prenatal intervention are unknown. We studied 40 fetuses referred for ultrasonography, examination of fetal urine, and possible therapy. We retrospectively assigned fetuses to a good prognosis group if fetal urine was hypotonic (sodium less than 100 mEq/L, chloride less than 90 mEq/L, osmolarity less than 210 mOsm/L) and there was no ultrasonographic evidence of dysplasia; we assigned fetuses to a poor prognosis group if even one criterion was abnormal. Survival was greater in the good prognosis group than in the poor prognosis group (81% vs 12.5%; 87% vs 30%, excluding abortions) (p less than 0.005). We then attempted to assess the efficacy of prenatal urinary decompression by comparing outcome within the good and poor prognosis groups. Survival with intervention was greater in both the good prognosis group and the poor prognosis group (89% vs 70% and 30% vs 0%). In 6 of the 8 survivors in the good prognosis group, severe oligohydramnios was reversed by decompression. We conclude the fetal urine electrolyte levels and ultrasonographic appear helpful in predicting residual fetal renal function and neonatal outcome and that prenatal decompression may prevent the development of fatal pulmonary hypoplasia.

Does an Expanding Fetal Abdominal Mass Produce Pulmonary Hypoplasia?

Fatal pulmonary hypoplasia has been related to multiple factors. In an effort to define which fetuses may benefit from prenatal intervention to prevent or reverse pulmonary hypoplasia, we studied the relative contribution of an enlarging abdominal in the fetus. We produced abdominal masses in fetal rabbits at 24 days gestation by two methods. In one group, a small cylindrical chip of Takasen, (a synthetic polymer that expands to 50 times its size in 1 week; Grobeast, Pop Art Co, Cleveland, OH) was inserted into the peritoneal cavity of the fetal rabbit; in another group, the blader neck was obstructed with a surgical clip. Amniotic fluid volume was restored at the surgical procedure. Sham-operated littermates served as controls. At cesarean delivery on day 30, fetal lung, liver, and body weights were measured, and the abdominal masses were quantitated by volume displacement of the removed or bladder. In both groups large abdominal masses of comparable size were produced. Newborns with the synthetic abdominal did not have significant pulmonary hypoplasia, but often had a prune belly deformity of the abdominal wall, whereas newborns with bladder obstruction had significant pulmonary hypoplasia. Liver weight was not significantly affected. We conclude that (1) a fetal abdominal does not independently produce pulmonary hypoplasia, possibly because the mass is relieved by distension of the abdominal wall rather than elevation of the diaphragm; (2) the pulmonary hypoplasia that occurs in bladder outlet obstruction is probably due to the associated oligohydramnios rather than the effect of the dilated urinary tract; and (3) prenatal decompression of an abdominal or dilated urinary tract is not justified to prevent pulmonary hypoplasia in the absence of oligohydramnios.

Does an expanding fetal abdominal mass produce pulmonary hypoplasia?

Fetal pulmonary hypoplasia has been related to multiple factors. In an effort to define which fetuses may benefit from prenatal intervention to prevent or reverse pulmonary hypoplasia, we studied the relative contribution of an enlarging abdominal mass in the fetus. We produced abdominal masses in fetal rabbits at 24 days gestation by two methods. In one group, a small cylindrical chip of Takasen, (a synthetic polymer that expands to 50 times its size in 1 week; Grobeast, Pop Art Co, Cleveland, OH) was inserted into the peritoneal cavity of the fetal rabbit; in another group, the bladder neck was obstructed with a surgical clip. Amniotic fluid volume was restored at the surgical procedure. Sham-operated littermates served as controls. At cesarean delivery on day 30, fetal lung, liver, and body weights were measured, and the abdominal masses were quantitated by volume displacement of the removed mass or bladder. In both groups large abdominal masses of comparable size were produced. Newborns with the synthetic abdominal mass did not have significant pulmonary hypoplasia, but often had a prune belly deformity of the abdominal wall, whereas newborns with bladder obstruction had significant pulmonary hypoplasia. Liver weight was not significantly affected. We conclude that a fetal abdominal mass does not independently produce pulmonary hypoplasia, possibly because the "mass effect" is relieved by distension of the abdominal wall rather than elevation of the diaphragm; the pulmonary hypoplasia that occurs in bladder outlet obstruction is probably due to the associated oligohydramnios rather than the mass effect of the dilated urinary tract; and prenatal decompression of an abdominal mass or dilated urinary tract is not justified to prevent pulmonary hypoplasia in the absence of oligohydramnios.