Early Human Development
Volume 88, Issue 1 , Pages 9-13, January 2012

Fetal counselling for surgical conditions

  • Kokila Lakhoo

      Affiliations

    • Corresponding Author InformationTel.: +44 1865 234197; fax: +44 1865 234211.

Article Outline

Abstract 

Foetal counselling is best achieved by a multidisciplinary team that can favourably influence the perinatal management of prenatally diagnosed anomalies and provide this information to prospective parents. Prenatal diagnosis has remarkably improved our understanding of surgically correctable congenital malformations. It has allowed us to influence the delivery of the baby, offer prenatal surgical management and discuss the options of termination of pregnancy for seriously handicapping or lethal conditions. Antenatal diagnosis has also defined an in utero mortality for some lesions such as diaphragmatic hernia and sacrococcygeal teratoma so that true outcomes can be measured. The limitation of in-utero diagnosis cannot be ignored. The aim of prenatal counselling is to provide information to prospective parents on foetal outcomes, possible interventions, appropriate setting, time and route of delivery and expected postnatal outcomes, immediate and long term.

Keyword: Prenatal counselling for surgical anomalies

 

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1. Introduction 

Expertise in surgical correction of congenital malformations, may favourably influence the perinatal management of prenatally diagnosed anomalies [1], [2]. A recent study [3] has confirmed the favourable impact of prenatal surgical consultation in influencing the site of delivery in 48% of cases, changing the mode of delivery by 10%, decision to terminate a pregnancy in 4.6% and change of diagnosis in 7%.

The diagnosis and management of complex foetal anomalies, require a multidisciplinary team encompassing obstetricians, neonatologists, genetecists, paediatricians, paediatric surgeons and occasional other specialist with expertise to deal with all the maternal and foetal complexities of a diagnosis of a structural defect[4].

Despite improvements in perinatal care serious birth defects still account for 20% of all deaths in the newborn period and an even greater percentage of serious morbidity later in infancy and childhood [5]. The major causes of congenital malformation are chromosomal abnormalities, mutant genes, multifactorial disorders and teratogenic agents.

Prenatal diagnosis has remarkably improved our understanding of surgically correctable congenital malformations. Routine ultrasound screening (18–20weeks in England and Wales) identifies anomalies and places these pregnancies in the high risk categories. High risk pregnancies maybe offered further invasive diagnostic investigations such as amniocentesis or chorionic villous sampling. Structural abnormalities difficult to define on ultrasound such as hindbrain lesions or in the presence of oligohydramnios are better imaged on ultra fast magnetic resonance imaging. With the increasing range of options and sophistication of diagnostic methods, parents today are faced with more information, choice and decisions than ever before, which can create as well as help to solve dilemmas.

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2. Specific surgical conditions 

2.1. Congenital Diaphragmatic Hernia (CDH) 

CDH accounts for 1 in 3000 live birth and challenges the neonatologist and paediatric surgeons in the management of this high-risk condition. Mortality remains high (more than 60%) when the ‘hidden’ mortality of in utero death and termination of pregnancy are taken into account. Lung hypoplasia and pulmonary hypertension account for most deaths in isolated CDH newborns. Associated anomalies (30%–40%) signify a grave prognosis with a survival rate of less than 10% [6].

In the UK most CDH are diagnosed at the 20week anomaly scan with a detection rate approaching 60%, although as early as 11weeks gestation has been reported. Magnetic resonance imaging (MRI) has a useful role in accurately differentiating CDH from cystic lung lesions and may be useful in measuring foetal lung volumes as a predictor of outcome. Cardiac anomalies (20%), chromosomal anomalies of trisomy 13 and 18 (20%) and urinary, gastrointestinal and neurological (33%) can co-exist with CDH and should be ruled out by offering the patient foetal echocardiogram, amniocentesis and detailed anomaly scan [6]. These associated anomalies and in isolated lesions, early detection, liver in the chest, polyhydramnios and foetal lung head ratio (LHR) of less than 1 are implicated as poor predictors of outcome. In these patients with poor prognostic signs foetal surgery for CDH over the last two decades has been disappointing, however benefit from foetal intervention with tracheal occlusion (FETO) awaits randomised studies [7]. Favourable outcomes in CDH with the use of antenatal steroids have not been resolved in the clinical settings. Elective delivery at a specialised centre is recommended with no benefit from caesarean section.

Postnatal management is aimed at reducing barotrauma to the hypoplastic lung by introducing high frequency oscillatory ventilation (HFOV) or permissive hypercapnea, and treating the severe pulmonary hypertension with nitric oxide. No clear benefits for CDH with ECMO (extra corporeal membrane oxygenation) have been concluded in a 2002 Cochrane ECMO study [8].

Surgery for CDH is no longer an emergency procedure. Delayed repair following stabilisation is employed in most paediatric surgical centres. Primary repair using the trans-abdominal route is achieved in 60–70% of patients with the rest requiring a prosthetic patch. Complications of sepsis or reherniation with prosthetic patch requiring revision are recorded in 50% of survivors [9]. Minimally invasive techniques have been successful in repairing diaphragmatic defects in ‘stable’ infants [10].

Long term survivors of CDH are reported to develop chronic respiratory insufficiency (48%) [11], gastro-oesophageal reflux (89%) [12] and neurodevelopment delay (30%) [9].

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3. Cystic lung lesions 

Congenital cystic adenomatoid malformations (CCAMs), bronchopulmonary sequestrations (BPS) or ‘hybrid’ lesions containing features of both are common cystic lung lesions noted on prenatal scan. Less common lung anomalies include bronchogenic cysts, congenital lobar emphysema and bronchial atresia. Congenital cystic lung lesions are rare anomalies with an incidence of 1 in 10000 to 1 in 35000 [13].

Prenatal detection rate of lung cysts at the routine 18–20week scan is almost 100% and may be the commonest mode of actual presentation. Most of these lesions are easily distinguished from congenital diaphragmatic hernia however sonographic features of CCAM or BPS are not sufficiently accurate and correlate poorly with histology [14]. Magnetic resonance imaging (MRI) though not routinely used, may provide better definition for this condition, however inaccuracies were reported in 11% of cases [13].

Bilateral disease and hydrops fetalis are indicators of poor outcome, whereas mediastinal shift, polyhydramnios and early detection are not poor prognostic signs [14]. In the absence of termination the natural foetal demise of antenatally diagnosed cystic lung disease is 28%. Spontaneous involution of cystic lung lesions can occur but complete postnatal resolution is rare, and apparent spontaneous ‘disappearance’ of antenatally-diagnosed lesions should be interpreted with care, as nearly half of these cases subsequently require surgery [15].

In only 10% of cases the need for foetal intervention arises. The spectrum of intervention includes simple centesis of amniotic fluid, thoracoamniotic shunt placement, percutaneous laser ablation and open foetal surgical resection. Maternal steroid administration has also been reported to have a beneficial effect on some CCAMs although the mechanism is unclear. A large cystic mass and hydrops in isolated cystic lung lesions is the only real indication for foetal intervention [14].

Normal vaginal delivery in recommended unless maternal condition indicates otherwise. Large lesion is predicted to become symptomatic shortly after birth (as high as 45% in some series) [16], thus delivery at a specialised centre would be appropriate, however smaller lesions are less likely to be symptomatic at birth and could be delivered at the referring institution with follow up in a paediatric surgery clinic [15].

Postnatal management is dictated by clinical status at birth. Symptomatic lesions require urgent radiological evaluation with chest radiograph and ideally CT scan followed by surgical excision. In asymptomatic cases postnatal investigation consists of chest CT scan within one month of birth, even if regression or resolution is noted on prenatal scanning. Plain radiography should not be relied upon since it will miss and underestimate many lesions [15], [17].

Surgical excision of postnatal asymptomatic lesions remains controversial, with some centres opting for conservative management [18].The approach to treating this asymptomatic group has evolved in some centres, whereby a CT scan is performed within one month post birth, followed by surgery before 6months of age due to the inherent risk of infection and malignant transformation. Small lesions, less than 1cm may be managed expectantly bearing in mind that the true resolution of these lesions is exceptional. Successful outcome of greater than 95% has been reported for these surgically managed asymptomatic lung lesions [13], [14], [15], [16].

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4. Abdominal wall defects 

Exomphalos and gastroschisis are both common but distinct abdominal wall defects with an unclear aetiology and a controversial prognosis. Attention may be drawn to their presence during the second trimester because of raised maternal serum alpha-fetoprotein level, or abnormal ultrasounds scan.

4.1. Exomphalos 

Exomphalos is characteristically a midline defect, at the insertion point of the umbilical cord, with a viable sac composed of amnion and peritoneum containing herniated abdominal contents. Incidence is known to be 1 in 4000 live births [19]. Associated major abnormalities which include trisomies 13, 18 and 21, Beckwith Wiedeman syndrome (macroglossia, gigantism, exomphalos), Pentology of Cantrell (sternal, pericardial, cardiac, abdominal wall and diaphragmatic defect), cardiac, gastrointestinal and renal abnormalities are noted in 60–70% of cases, thus karyotyping, in addition to detailed sonographic review and foetal echocardiogram is essential for complete prenatal screening. Foetal intervention is unlikely in this condition. If termination is not considered, normal vaginal delivery at a centre with neonatal surgical expertise is recommended and delivery by caesarean section only reserved for large exomphalos with exteriorised liver to prevent damage [20].

Surgical repair includes primary closure or a staged repair with a silo for giant defects. Occasionally in vulnerable infants with severe pulmonary hypoplasia or complex cardiac abnormalities the exomphalos may be left intact and allowed to slowly granulate and epithelialise by application of antiseptic solution [21]. Postnatal morbidity occurs in 5–10% of cases. Malrotation and adhesive bowel obstruction do contribute to mortality in isolated exomphalos however the majority of these children survive to live normal lives [20], [21].

4.2. Gastroschisis 

Gastroschisis is an isolated lesion that usually occurs on the right side of the umbilical defect with evisceration of the abdominal contents directly into the amniotic cavity. The incidence is increasing from 1.66 per 10000 births to 4.6 per 10000 births affecting mainly young mothers typically less than 20years old. Associated anomalies are noted in only 5–24% of cases with bowel atresia the most common co-existing abnormality [22]. On prenatal scan with a detection rate of 100%, the bowel appears to be free floating, and the loops may appear to be thickened due to damage by amniotic fluid exposure causing a ‘peel’ formation. Dilated loops of bowel may be seen from obstruction secondary to protrusion from a defect or atresia due to intestinal ischaemia [23].

Predicting outcome in foetuses with gastroschisis based on prenatal ultrasound finding remains a challenge. There is some evidence that maximum small bowel diameter may be predictive, however thickened matted bowel and Doppler measurements of the superior mesenteric artery are not accurate predictors of outcome. To reduce the rate of third trimester foetal loss serial ultrasounds are performed to monitor the development of bowel obstruction and delivery around 37weeks recommended at a centre with neonatal surgical expertise [24].

Recent study by Logghe et al. has challenged elective preterm delivery with a randomised control trial [25]. Delivery by caesarean section has no advantage to normal vaginal route. Despite efforts to plan elective delivery, 50% of cases will require emergency caesarean section due to development of foetal distress [26]. Various methods of postnatal surgical repair include the traditional primary closure, reduction of bowel without anaesthesia [27], reduction by preformed silo [28], or by means of a traditional silo. Coexisting intestinal atresia could be repaired by primary anastamosis or staged with stoma formation. Variation in achieving full enteral feeding due to prolonged gut dysmotility is expected in all cases.

The long term outcome in gastroschisis is dependent on the condition of the bowel. In uncomplicated cases the outcome is excellent in more than 90% of cases [29]. The mortality of live born infants is 5% with further 5% suffering short bowel syndrome and 10% requiring surgery for adhesive bowel obstruction [29]. Late third trimester foetal loss should always be mentioned during foetal counselling.

4.2.1. Tracheo-oesophageal fistula (TOF) and oesophageal atresia (OA) 

Repair of TOF/OA is a condition, which measures the skill of paediatric surgeons from trainee to independent surgeon. The incidence is estimated at 1 in 3000 births. Prenatally, the condition may be suspected from maternal polyhydramnios and absence of a foetal stomach bubble at the 20-week anomaly scan. Prenatal scan diagnosis of TOF/OA is estimated to be less than 42% sensitive with a positive predicted value of 56% [30]. Additional diagnostic clues are provided by associated anomalies such as trisomy (13, 18, 21), VACTERAL sequence (vertebral, anorectal, cardiac, tracheo-oesophageal, renal, limbs) and CHARGE association (coloboma, heart defects, atresia choanae, retarded development, genital hypoplasia, ear abnormality). These associated anomalies are present in more than 50% of cases and worsen the prognosis, thus prenatal karyotyping is essential [31]. Duodenal atresia may coexist with TOF/OA. The risk of recurrence in subsequent pregnancies for isolated TOF/OA is less than 1% [32]. Delivery is advised to be at specialised centre with neonatal surgical input.

Postnatal surgical management is dependent on the size and condition of the baby, length of oesophageal gap and associated anomalies. Primary repair of the oesophagus is the treatment of choice, however if not achieved, staged repair with upper oesophageal pouch care and gastrostomy or organ replacement with stomach or large bowel are other options. Associated anomalies require evaluation and treatment. Minimally invasive thoracoscopic approach to the repair of TOF may be offered by advanced paediatric endosurgical centres[33], [34].

Early outcome of a high leak rate and oesophageal stricture requiring dilatation in 50% of cases are expected where the anastamosis of the oesophagus is created under tension [35].

Long term outcome is indicated by improved perinatal management and inherent structural and functional defects in the trachea and oesophagus. In early life, growth of the child is reported to be below the 25 centile in 50% of cases, respiratory symptoms in two thirds of TOF/OA and gastro-oesophageal reflux recorded in 50% of patients [36]. Quality of life is better in the isolated group with successful primary repair as compared to those with associated anomalies and delayed repair.

4.3. Gastrointestinal lesions 

The presence of dilated loops of bowel (>15mm in length and 7mm in diameter) on prenatal ultrasound scan is indicative of bowel obstruction.

Duodenal atresia has a characteristic ‘double bubble’ appearance on prenatal scan, resulting from the simultaneous dilatation of the stomach and proximal duodenum. Detection rate on second trimester anomaly scan is almost 100% in the presence of polyhydramnios and the ‘double bubble’ sign. Associated anomalies are present in approximately 50% of cases with most notably trisomy 21 in 30% of cases cardiac anomalies in 20% and the presence of the VACTERL association (vertebral, anorectal, cardiac, tracheo-oesophageal, renal and limbs) [37].

The incidence of duodenal atresia is 1 in 5000 live birth. The postnatal survival rate is >95% with associated anomalies, low birth weight and prematurity contributing to the <5% mortality. Temporary delay in enteral feeding occurs due to the dysmotility in the dilated stomach and duodenum [37], [38].

There are many bowel abnormalities which may be noted on prenatal scanning (dilated bowel, ascites, cystic masses, hyperparistalsis, poyhydramnios and echogenic bowel) [37], however none is absolutely predictive of postnatal outcome. Patients with obstruction frequently have findings (especially in the third trimester) of bowel dilatation, polyhydramnios and hyperparistalsis, but ultrasound is much less sensitive in diagnosing large bowel anomalies than those in small bowel. Since the large bowel is mostly a reservoir, with no physiologic function in utero, defects in this region such as anorectal malformations or Hirshsprung's disease are very difficult to detect. Bowel dilatation and echogenic bowel may be associated cystic fibrosis, therefore all such foetuses should undergo postnatal evaluation for this disease Prenatally diagnosed small bowel atresia does not select for a group with a worse prognosis and survival rates are 95–100% [38].

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5. Abdominal cysts 

Abdominal cystic lesions are not uncommonly diagnosed at antenatal ultrasound (US) examination. A cystic mass identified in this way may represent a normal structural variant or a pathological entity requiring surgical intervention postnatally. Despite increasingly sophisticated equipment some congenital anomalies have significant false positive rates on US and foetal cystic abdominal masses in particular can be difficult to diagnose accurately [39]. Excluding cysts of renal origin the differential diagnosis includes ovarian cysts, enteric duplication cysts, meconium pseudocyst, mesenteric cysts and choledochal cysts. Less common diagnoses include extra-lobar pulmonary sequestration and pancreatic, splenic, urachal and adrenal cysts. Almost all cysts are benign and many are self limiting, however these cysts create a high level of anxiety for the prospective parents, especially suspected adrenal cyst. Regular antenatal consultation and foetal counselling by the appropriate team may reduce parental anxiety levels. There is a very small role for foetal intervention. Resolution of these cysts was reported in 25% of cases and 30% came to surgical intervention [39]. Postnatal imaging is essential.

5.1. Sacrococcygeal teratoma 

Sacrococcygeal teratoma (SCT) is the commonest neonatal tumour accounting for 1 in 35000 to 40000 births. Four types have been defined [40]. Doppler ultrasound is the diagnostic tool, however foetal MRI provides better definition of the intrapelvic component [41]. SCT is a highly vascular tumour and the foetus may develop high cardiac output failure, anaemia and ultimately hydrops with a mortality of almost 100% [42]. Foetal treatment of tumour resection or ablation of feeding vessel has been attempted in hydropic patients [43]. Caesarean section may be offered to patients with large tumours to avoid the risk of bleeding during delivery. Postnatal outcomes following surgery in the mainly extrapelvic lesions are favourable however intra pelvic tumours may present with urological problems and less favourable outcomes [44]. Long term follow up with alpha feto protein and serial pelvic ultrasounds is mandatory to exclude recurrence of the disease.

5.2. Renal anomalies 

Urogenital abnormalities are among the commonest disorders seen in the perinatal period and account for almost 20% of all prenatally diagnosed anomalies [45]. The routine use of antenatal ultrasound scans has resulted in the early detection of these conditions and in selected cases has led to the development of management strategies including foetal intervention aimed at preservation of renal function. Two major issues are the indications for intervention in bladder outlet obstruction and early pyeloplasty in infancy in cases with hydronephrosis [46].

Prenatal evaluation of a dilated urinary tract is based on serial ultrasound scans as well as measurement of urinary electrolytes. Ultrasonography provides measurements of the renal pelvis, assessment of the renal parenchyma as well as the detection of cysts in the cortex. In severe disease, lack of amniotic fluid may make ultrasound assessment of the renal tract difficult and MRI may be helpful. Oligohydramnios is indicative of poor renal function and poor prognosis owing to the associated pulmonary hypoplasia. Urogenital anomalies co-exist with many other congenital abnormalities and amniocentesis should be offered in appropriate cases. It is estimated that 3% of infants will have an abnormality of the urogenital system and half of these will require some form of surgical intervention [47].

5.3. Upper urinary tract obstruction 

Antenatal hydronephrosis accounts for 0.6–0.65% of pregnancies [48]. The most common cause of prenatal hydronephrosis is pelviureteric junction obstruction (PUJ), others being transient hydronephrosis, physiological hydronephrosis, multicystic kidney, posterior urethral valves, ureterocoele, ectopic ureter, etc. The prognosis of antenatally diagnosed hydronephrosis in unilateral disease and in renal pelvic diameter of <10mm, is excellent [49]. Spontaneous resolution is noted in 20% of patients at birth and 80% at 3years of age [48]. Only 17% of prenatally diagnosed hydronephrosis needs surgical intervention. Postnatal management of hydronephrosis requires ultrasound at birth and at 1month of age, and further evaluation with radiology and scintigraphy if an abnormality is suspected. The non-operative treatment of antenatally detected hydronephrosis has been carefully monitored over a 17year period and from an analysis of 6 patient series the conclusion is that this approach is safe [50].

5.4. Lower urinary tract obstruction 

Posterior urethral valves (PUV) are the most common cause for lower urinary tract obstruction in boys with an incidence of 1 in 2000 to 4000 live male births. The diagnosis of PUV is suspected on the prenatal ultrasound finding of bilateral hydronephrosis associated with a thickened bladder and decreased amniotic fluid volume [51]. Serial foetal urine analysis may provide prognostic information on renal function. Prenatal diagnosis for patients with PUV is a poor prognostic sign with 64% incidence of renal failure and transient pulmonary failure, compared to 33% in the postnatally diagnosed patients[52]. Pulmonary hypoplasia secondary to oligohydramnios largely contributes to the morbidity and mortality from foetal urethral obstruction. Outcomes of foetal intervention with vescicoamniotic shunting [53], [54] or foetal cystoscopic ablation of urethal valve [54]are still under review and await a multicenter trial.

Postnatal management includes ultrasound confirmation of the diagnosis, bladder drainage via a suprapubic or urethral route and contrast imaging of the urethra. Either primary PUV ablation, vescicostomy or ureterostomy is a postnatal surgical option. The overall outcome from this disease is unfavourable [55].

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6. Conclusion 

The diagnosis and management of complex foetal anomalies, require a team effort by obstetricians, neonatologists, genetecists, paediatricians and paediatric surgeons to deal with all the maternal and foetal complexities of a diagnosis of a structural defect. This team should be able to provide information to prospective parents on foetal outcomes, possible interventions, appropriate setting, time and route of delivery and expected postnatal outcomes. The role of the surgical consultant, in this team, is to present information regarding the prenatal and postnatal natural history of an anomaly, its surgical management, and the long term outcome.

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7. Key guidelines 


1.The aim of prenatal diagnosis and testing is to have 100% accuracy without foetal loss or injury and no maternal risk.

2.The care of patients with surgically correctable defects can now be planned prenatally with the collaborative effort of obstetricians, geneticists, neonatologists and paediatric surgeons.

3.Essential to prenatal counselling is the understanding of the specific surgical condition's prenatal natural history, the limitations of prenatal diagnosis and the detection of associated anomalies.

4.The risks and indications of foetal intervention programmes should be fully understood.

5.Prenatal counselling should provide information to prospective parents on foetal outcomes, possible interventions, appropriate setting, time and route of delivery and expected postnatal outcomes, immediate and long term.

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8. Research directions 


1.Research into new markers for chromosomal abnormalities is ongoing. The foetal nasal bone is one such example, which may assist, in detecting babies with chromosomal abnormalities.

2.Rapid detection techniques versus traditional cultures for karyotyping are currently under debate at present.

3.Three-dimensional images from new ultrasound machines may have a useful role in diagnosis and assessment of facial deformities such as cleft lip and palate.

4.Magnetic resonance imaging (MRI) may assist in better defining some lesions difficult to view on conventional prenatal scanning.

5.Pschycological studies on the impact of foetal counselling needs further evaluation.

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Conflict of interest 

There is no conflict of interest in this manuscript.

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PII: S0378-3782(11)00350-1

doi:10.1016/j.earlhumdev.2011.11.004

Early Human Development
Volume 88, Issue 1 , Pages 9-13, January 2012

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