Long-term outcome in patients with prenatally diagnosed cystic lung disease: special reference to ventilation and perfusion scan in the affected lung

Background/Purpose

The natural history of cystic lung disease (CLD) such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration has been altered by the advent of prenatal diagnosis. Although recent advances including fetal therapy have gradually improved outcome, the long-term course and the function of the residual lung have not been well clarified.

Methods

Twenty-two patients with CLD who had been prenatally diagnosed and treated between 1990 and 2004 were reviewed. The clinical outcome and growth measurements were established, and, where possible, all infants underwent ventilation and perfusion lung scan.

Results

Mediastinal shift was present in 14 fetuses. Fetal hydrops was present in 5 fetuses. Antenatal intervention was performed for hydrops in 2 fetuses (cyst-amniotic shunt and aspiration). Twenty-one infants underwent appropriate excisional surgery. Final diagnosis included CCAM (n = 12) and pulmonary sequestration (n = 7). No late death was observed. Common complications were failure to thrive (n = 5), frequent respiratory tract infection (n = 4), and asthmatic attack (n = 4). A significant decrease in lung ventilation and perfusion on the affected side was observed in patients with hydrops, lobectomy, and CCAM.

Conclusion

Long-term follow-up including respiratory care and growth assessment should be performed in prenatally diagnosed patients with CLD, especially those who present with hydrops.     

Bronchial atresia: the hidden pathology within a spectrum of prenatally diagnosed lung masses

Purpose

This study was aimed at determining whether different congenital lung masses represent diverse manifestations of a single developmental abnormality associated with fetal airway obstruction.

Methods

We conducted a 3-year retrospective review of patients who underwent surgical resection of a prenatally diagnosed lung mass. Prenatal imaging was used to define mass position and its effect on adjacent organs. Lung specimens were examined through careful full-specimen microdissections, as well as by plain and contrast roentgenograms.

Results

Twenty-five patients underwent lung resection during this study period. Based on the final pathology reports, 56% were congenital cystic adenomatoid malformations, 12% were congenital lobar emphysemas, 8% were bronchopulmonary sequestrations, and 24% had features of both cystic adenomatoid malformation and bronchopulmonary sequestrations. No bronchogenic cysts were present in this series. Overall, bronchial atresia was identified in 77% of the examined specimens (n = 22) and was associated with all types of lung malformations.

Conclusions

Bronchial atresia is a common, unrecognized component of prenatally diagnosed congenital cystic adenomatoid malformations, bronchopulmonary sequestrations, congenital lobar emphysemas, and lesions of mixed pathology. Most congenital lung masses may be part of a spectrum of anomalies linked to obstruction of the developing fetal airway as an underlying component in their pathogenesis.     

Outcomes after postnatal resection of prenatally diagnosed asymptomatic cystic lung lesions

Background

Symptomatic congenital lung lesions require surgical resection, but the management of asymptomatic lung lesions is controversial. Some surgeons advocate observation because of concerns about potential operative morbidity and mortality, as well as a lack of long-term follow-up information. On the other hand, malignant degeneration, pneumonia, and pneumothorax are known consequences of cystic lung lesions. This study aims to assess the safety of resection for asymptomatic lung lesions that were diagnosed before birth.

Methods

A retrospective review of all patients with prenatally diagnosed lung lesions at Children's Hospital of Philadelphia (Philadelphia, Penn) was performed from 1996 to 2005. The perioperative course of patients who were asymptomatic was analyzed.

Results

One hundred five complete records of children with asymptomatic lesions were reviewed. Overall mortality was 0% and morbidity was 6.7% including 2.9% significant postoperative air leak and 3.8% transfusion requirement. Nine patients had a pathologic diagnosis that differed from preoperative radiological findings, and 9 patients had additional pathologic findings.

Conclusion

This series demonstrates that surgery can be performed safely on patients who were asymptomatic with congenital cystic adenomatoid malformation of the lung and other types of lung lesions with no mortality and minimal morbidity. The frequency of disparate pathologic diagnoses and the potential for development of malignancy and other complications support the argument for early resection.     

Familial case of prenatally diagnosed intralobar and extralobar sequestrations with cystadenomatoid change

Hybrid lesions are part of a spectrum of rare pulmonary diseases that are characterized as having elements of both congenital pulmonary airway malformation and bronchopulmonary sequestration. Fetal thoracic masses arise from alterations during lung development that are separated by timing of the inciting event and are often associated with an underlying degree of bronchial atresia. There are a handful of documented reports of sequestrations occurring in siblings, but no known reports of prenatally diagnosed lesions occurring in families. We present a case of 2 siblings diagnosed prenatally with fetal thoracic lesions who underwent postnatal resection revealing hybrid lesions on pathologic examination. Newer radiologic techniques have increased our ability to detect these masses prenatally, as well as follow them throughout pregnancy to determine their natural history. Ongoing laboratory investigation into the etiology of congenital lung lesions has brought forth more questions and suggested a familial component at a cellular level that has not yet been fully discovered. We reviewed the current literature of factors contributing to the development of congenital lung lesions and suggest that there is a familial link in certain patient populations where screening may be indicated.     

Intraabdominal pulmonary sequestration. A case series and review of the literature

Pulmonary sequestrations are infrequent congenital malformations, and very rarely they are located below the diaphragm, particularly in the left suprarenal area. Prenatal detection is available. Differential diagnosis includes lesions such as enteric duplication cysts and neuroblastomas. Between 1999 and 2002, we operated on 4 patients with intraabdominal pulmonary sequestration. This series is presented here, with a review of the literature and current guidelines for the management of these unusual lesions.     

Two cases of prenatally diagnosed congenital lobar emphysema caused by lobar bronchial atresia

Bronchial atresia is a rare congenital anomaly, and neonatal presentation is extremely rare. Bronchial atresia often affects segmental or subsegmental bronchi, and bronchial atresia of a lobar bronchus is also rare. We report 2 cases of prenatally diagnosed congenital lobar emphysema caused by bronchial atresia at the left upper lobar bronchus. Three-dimensional computed tomography bronchoscopy was effective to demonstrate accurate anatomical changes of the bronchus.     

Current outcome of antenally diagnosed cystic lung disease

Background/purpose

The natural history of parenchymal lung lesions such as congenital cystic adenomatoid malformation (CCAM) and pulmonary sequestration (PS) has been altered by the advent of antenatal ultrasonography. Initial reports were characterized by a high (about 30%) incidence of adverse features (eg, hydrops) and a poor outcome and did not accord with our recent experience. The authors have reviewed the outcome of fetuses that had been diagnosed in a large tertiary referral fetal medicine unit with the aim of delineating current experience. The term cystic lung disease was used throughout to avoid unjustifiable histologic precision.

Methods

The scans of all fetuses that had been diagnosed with cystic lung disease between January 1995 and July 2001 were reviewed. The outcome of each pregnancy was established, and, where possible, all infants underwent appropriate investigations, including thoracic computed tomography (CT) scans.

Results

Sixty-seven fetuses had a cystic lung abnormality diagnosed from January 1995 to July 2001. The median (range) age at diagnosis was 21 (19 to 28) weeks. The lesion was right sided in 29 (43%), left in 36 (54%), and bilateral in 2 (3%); it was characterized as dominantly macrocystic in 27 (40%), microcystic in 35 (52%), and mixed in 5 (8%). Mediastinal shift was present in 30 fetuses (45%). Severe signs of fetal distress (eg, hydrops) were present in 5 fetuses (7%). Antenatal intervention was performed in 4 fetuses (thoraco-amniotic shunts [n = 3] and percutaneous intrauterine laser therapy [n = 1]). Sixty-four (96%) of the fetuses were born alive. There was 1 termination of pregnancy and 2 intrauterine deaths (all severe microcystic lesions). Forty-two infants (63%) underwent thoracotomy and appropriate excisional surgery at a median of 7.5 months (range, 1 day to 34 months). Two infants (which included the fetus having intrauterine laser therapy) died early in the postnatal period. Both were large microcystic lesions and had antenatal features of severe fetal distress. Twelve infants were investigated in the postnatal period but did not undergo surgery. Ten infants were not appropriately investigated or were lost to follow-up. Histologic examination showed definitive diagnostic features of CCAM (n = 25) or PS (n = 6). Other lesions with hybrid features of both were also seen (n = 11). There was a degree of correlation between antenatal ultrasound features (size of cyst [P = .03], in-utero behavior [P = .06], mediastinal shift [P = 0.05]) and the need for surgery but not with the final histologic diagnosis. Surgical excision was required in 45% of lesions showing late-gestation “resolution.”

Conclusions

Antenatally diagnosed “cystic lung disease” has an excellent prognosis in the absence of signs of severe fetal distress. The need for surgery should be based on appropriate postnatal investigations (eg, CT scans), rather than on antenatal behavior.     

Chronic inflammation in congenital cystic adenomatoid malformations. An underestimated risk factor?

Purpose

Congenital cystic malformations of the lung are more frequently diagnosed before birth, but guidelines for surgical management of asymptomatic cases are lacking. The aim of this article is to review our 10-year results with antenatally diagnosed congenital cystic adenomatoid malformations (CCAMs) to debate indications for early postnatal surgical management in asymptomatic patients.

Method

Twenty-four cases were reviewed; of these, 18 were operated on before 15 days of life for respiratory distress or mediastinal shift, whereas 6 were submitted to elective surgery at 3 months of age.

Results

Twenty lobectomies and 4 atypical resections were performed. Two of the latter required a second surgery for incomplete primary perinatal resection. No postsurgical complications were reported. Nineteen (19/24) of the resected specimens showed signs of chronic inflammation. In the perinatal period, 100% (8 cases) of CCAM type II and 50% (8 cases) of CCAM type I resulted to be inflamed. Of the asymptomatic cases, 50% (3/6) were also found to be affected. No infections were detected at bacteriologic culture and bacterial debris was stained in 3 specimens.

Conclusion

In this series, a 79% incidence of pulmonary inflammation was detected. The CCAM type II resulted to be always involved in this process of inflammation. This was an unexpected finding, particularly in cases without mediastinal shift or respiratory distress. In light of these results, early postnatal treatment, at around 3 to 6 months of age, could be considered even in asymptomatic patients.     

四例慢性阻塞性肺病行单肺移植的体会

目的 总结同种异体肺移植治疗慢性阻塞性肺病的体会。方法 4例慢性阻塞性肺病患者，1例为双肺弥漫性肺气肿，另3例为双肺重度弥漫性肺气肿并部分相互融合成肺大疱，但肺大疱占据胸腔不足1／3。4例均接受尸体供肺移植，为双肺弥漫性肺气肿的1例接受右肺移植，弥漫性肺气肿并部分相互融合成肺大疱的3例接受左肺移植，此3例分别于术中（1例）和术后（2例）接受右肺减容术。术后采用他克莫司、霉酚酸酯及泼尼松预防排斥反应，同时给予达利珠单抗或抗胸腺细胞球蛋白进行诱导治疗。结果4例手术经过顺利，1例术后第5d发生急性排斥反应，经甲泼尼龙冲击治疗逆转。2例受者顺利出院，并存活至今，最长1例存活已超过2年。2例分别于肺移植术后第74d、77d死亡。结论 终末期双肺弥漫性肺气肿可行单侧肺移植治疗，如果对侧肺有重度弥漫性肺气肿，特别是部分融合成大疱易破裂者，可同期期行对侧肺减容。     

Prenatal diagnosis and management of abdominal diseases in pediatric surgery

Purpose

The aim of this study was to investigate the prenatal courses and management of abdominal surgical diseases.

Methods

Of the 327 patients registered with our fetal treatment board since March 2002, 83 fetuses referred to the surgical department were enrolled for the current study. The prenatal diagnosis, sequential fetal images, and perinatal courses of these cases were reviewed retrospectively.

Results

Of the 83 cases, abdominal diseases were suspected in 34, lung and thoracic diseases in 25, genitourinary diseases in 12, and other anomalies in 12. Meconium peritonitis (MP), intestinal obstruction, and abdominal wall defects accounted for approximately 65% of the abdominal diseases. Five patients with prenatally diagnosed lung diseases underwent fetal surgical intervention, and 17 of the 22 liveborn patients survived. In contrast, none of the patients with prenatally diagnosed abdominal anomalies underwent fetal surgical intervention, yet, 23 of the 24 liveborn patients survived. However, preterm labor and hydrops were seen frequently in the patients with giant cystic MP, suggesting a fetal critical condition.

Conclusions

Although the clinical outcome of abdominal diseases seemed favorable with postnatal treatment, the current results suggested the occurrence of hidden mortality in utero and the potential need for fetal intervention for some abdominal conditions, such as MP.     

肺移植治疗终末期肺疾病的单中心十年经验总结

目的 探讨肺移植治疗终末期肺疾病的效果,并对单中心肺移植的经验进行总结.方法 回顾2003年1月至2012年12月间52例肺移植病例的临床资料,受者年龄为24～76岁,≥65岁者13例;受者原发病主要为肺气肿33例(63.5％)及特发性肺间质纤维化8例(15.4％).术后对所有受者的并发症发生率、死亡情况及存活率进行分析.结果 供肺缺血时间＞6 h者28例(53.8％),其中缺血超过10 h者20例(38.5％).术后出现吻合口狭窄3例(5.8％),住院期间发生细菌感染14例(26.9％),真菌感染13例(25.0％),巨细胞病毒性肺炎1例(1.9％).发生急性排斥反应20例(38.5％),经甲泼尼龙冲击治疗3d后均逆转;发生慢性排斥反应7例(13.5％),其中2例接受再次肺移植后效果良好,3例调整免疫抑制方案后缓解,2例死亡.术后1年内死亡9例(17.3％),术后1、3和5年的总体累积存活率分别为81.4％,54.5％和30.9％.结论 肺移植是治疗终末期肺疾病的有效方法,恰当的病例选择、良好的肺保护、正确的围手术期处理及系统的术后管理是肺移植成功的关键.     

Differential risk for neonatal surgical airway intervention in prenatally diagnosed neck masses

Purpose

We aimed to identify risk factors for neonatal surgical airway intervention among fetuses with prenatally diagnosed cervical masses.

Methods

An 8-year retrospective review identified 23 consecutive patients with a prenatal diagnosis of a neck mass, managed at a single tertiary center. Variables analyzed included anticipated diagnosis, extent of the mass, need for any surgical airway intervention in the neonatal period, final histopathology data, and survival. Statistical analysis was based on the Fisher and Fisher-Freeman-Halton exact tests (significance set at P ≤ .05) and exact 95% confidence intervals for risk differences.

Results

Eight patients underwent termination of pregnancy or were lost to follow-up. The imaging-based prenatal diagnosis was confirmed postnatally in 93% (14/15) of the remaining patients. Final diagnoses included lymphatic malformation (8), teratoma (6), and esophageal duplication (1). Teratomas were associated with a significantly higher risk for neonatal airway intervention than lymphatic malformations (67% vs 11%, P = .02). The majority of such procedures were performed under ex utero intrapartum treatment. Survival was 93% (14/15).

Conclusions

Cervical teratomas are significantly more likely to demand surgical airway intervention in the neonate, typically under ex utero intrapartum treatment, than cervical lymphatic malformations. These findings should be considered in the prenatal counseling for fetal cervical masses.     

The outcome of prenatally diagnosed renal tumors

PURPOSE: We assessed the incidence of perinatal morbidity and evaluated the outcome in children with prenatally diagnosed renal tumors in a retrospective multicenter study. MATERIALS AND METHODS: A review of the records of patients from 20 institutions identified 28 children with prenatally diagnosed renal tumors. Prenatal findings, clinical charts, and radiological, surgical and pathological reports were reviewed in this study. RESULTS: There were 26 congenital mesoblastic nephromas and 2 Wilms tumors. One or more complications were identified in 20 of the 28 cases (71%) during the perinatal period. Polyhydramnios was observed in 11 fetuses (39%), 2 presented with hydrops fetalis and 7 presented in acute fetal distress requiring emergency cesarean section, of which 1 died in utero before delivery. Median gestational age of the 27 neonates born alive was 35 weeks (range 29 to 39), including 13 (46%) who were pre-term (less than 34 weeks of gestation). Complications at birth included hemodynamic instability in 3 newborns, of whom 2 underwent emergency surgery, respiratory distress syndrome in 8 (30%) and hypertension in 6 (22%). Surgical complications occurred in 7 patients (26%), including tumor rupture in 1 and intraoperative bleeding with postoperative death in 1. At a median followup of 42 months (range 2 to 105) 26 of the 27 children were in complete remission. CONCLUSIONS: Fetal renal tumors have an excellent oncological outcome but a high risk of perinatal complications. Prenatal diagnosis should allow planning the delivery at a pediatric tertiary care center to avoid a potentially life threatening condition in neonates in the first hours of life.     

右肺中叶疾病的诊断与外科治疗

目的　探讨右肺中叶疾病 (简称中叶疾病 )的诊断与外科治疗的特殊性和规律性 ,为临床诊治提供依据。　方法　回顾分析我院 1989年 1月～ 2 0 0 2年 4月手术治疗中叶疾病 16 3例的临床资料及随访结果。　结果　 16 3例中 ,78例 (47 9% )为恶性肿瘤 ,85例 (5 2 1% )为良性疾病 ;<5 0岁者 91例 ,81 3% (74例 )为良性疾病 ,显著高于恶性肿瘤的发生率 (P <0 0 1) ,≥ 5 0岁者 72例 ,84 7%(6 1例 )为恶性肿瘤 ,显著高于良性疾病的发生率 (P <0 0 1) ;11例结核患者中 8例 (72 7% )合并支气管扩张。术后除 1例因呼吸衰竭死亡外均康复出院 ;7例 (4 3% )叶间积液 ,穿刺抽液后恢复。恶性肿瘤患者的 1、3、5年生存率 ,Ⅰ、Ⅱ期分别为 88 4 % (38/ 4 3)、6 2 8% (2 7/ 4 3)、5 1 2 % (2 2 / 4 3) ,Ⅲ、Ⅳ期分别为 76 5 % (2 6 / 34)、4 1 2 % (14 / 34)、14 7% (5 / 34)。 4例楔形切除恶性肿瘤者均术后 1年内复发 ,1例接受再次手术切除中叶及下叶者术后 38个月死于全身衰竭 ,良性病变无复发。结论　 (1)对中叶疾病应重视肺癌的可能 ,尤其对 5 0岁以上者 ;(2 )中叶疾病性质难以明确时 ,宜剖胸探查 ;(3)中叶结核多合并支气管扩张 ,保守治疗效果不佳 ;(4)单纯中叶切除术后在右侧第 4肋间锁骨中线内侧 2cm处放置     

Postnatal management for prenatally diagnosed choledochal cysts

Purpose

The aim of this study was to determine an appropriate postnatal management plan for prenatally diagnosed congenital biliary dilatation (CBD).

Methods

Between 1962 and 2002, 5 (5.9 %) of 85 patients had CBD diagnosed prenatally and were examined clinically. Of these 5 patients, 2 (group A) underwent delayed primary definitive surgery after percutaneous transhepatic cholangiodrainage (PTCD), 1 (group B) underwent early definitive surgery in the neonatal period, and 2 (group C) underwent delayed primary definitive surgery without PTCD in early infancy (within 6 months after birth). The clinical data, operative findings, intra- and postoperative complications, and follow-up were evaluated in these 3 groups.

Results

There were no postoperative complications, such as catheter-related complications, in group A. However, there was adhesion around the choledochal cyst, and the operation was therefore difficult in group A. The diameter of the anastomosis in the hepaticojejunostomy was small, and the cyst wall was thin in group B. Consequently, anastomotic leakage of the hepaticojejunostomy occurred in group B. Neither operative nor postoperative complications such as anastomotic leakage or stenosis occurred in group C. Slight fibrosis of Glisson’s sheath was seen in 2 patients of groups A and C. No liver cirrhosis was seen in any group.

Conclusions

The authors propose that asymptomatic patients should undergo elective definitive surgery by 6 months of age. For symptomatic patients, especially when a differential diagnosis of type I cystic biliary atresia is doubtful, early definitive surgery is needed before 2 months of age. PTCD appears to be indicated only under certain circumstances, and delayed primary definitive surgery should be performed as early as possible thereafter.     

CT引导下经皮肺穿刺活检在肺部疾病诊断中的应用

目的探讨CT引导下经皮肺穿刺活检术的临床应用价值。方法连续收集105例肺部病变患者,分为局灶结节组（直径≤3cm）、局灶团块组（直径〉3cm）及弥漫性病变组。在CT引导下行活检针穿刺活检,穿刺活检病理结果与手术病理或临床随访结果对照。结果对101例患者完成活检,穿刺活检成功率为96.19%（101/105）;其中局灶结节组33例,局灶团块组59例,弥漫性病变组9例。与手术病理或临床随访结果对照,总诊断符合率为92.08%（93/101）,3组的诊断符合率分别为87.88%（29/33）,93.22%（55/59）及100%（9/9）,各组间诊断符合率差异均无统计学意义（P均〉0.05）;总并发症发生率为12.38%（13/105）。Logistic回归分析显示,病灶大小是出血的影响因素（χ2=7.77,P=0.005）,病灶大小和病灶周围有无肺气肿是气胸的影响因素（P〈0.001）。结论 CT引导下经皮肺穿刺成功率及诊断符合率较高,并发症少,是一种具有较高实用性和安全性的活检取材方法。病灶直径≤3cm及病灶周围广泛肺气肿可增大并发症风险。     

Current outcome of antenatally diagnosed exomphalos: an 11 year review

Purpose

The aim of this study was to determine the outcome of antenatally diagnosed exomphalos.

Methods

The database of a tertiary referral Fetal Medicine Centre was searched for all cases of antenatally diagnosed exomphalos between January 1991 and December 2002. Patients, general practitioners, and hospitals were contacted for outcome details.

Results

In total, 445 cases of exomphalos were identified. In 250 (56%) cases, the fetal karyotype was abnormal (group A), in 135 (30%) cases, the karyotype was normal (group B), and in 60 (14%) cases, karyotyping was declined (group C). In group A, there were 248 (99%) terminations of pregnancy (TOP) or fetal deaths and 2 live births. In group B, 74 (54%) fetuses had other structural anomalies; 82 (61%) pregnancies resulted in TOP or fetal death, 42 (31%) in live births, and 11 (8%) were lost to follow-up. In group C, 38 (63%) fetuses had other structural anomalies; 41 (69%) pregnancies resulted in TOP or fetal death, 11 (18%) in live births, and 8 (13%) were lost to follow-up. Of the 55 live births, 11 died preoperatively and 44 had surgery. There were no postoperative deaths.

Conclusions

Less than 10% of the antenatal diagnostic workload reached operative repair. In our unit, these babies are a highly selected group, which is a factor in the high postoperative survival.     

Prenatal pulmonary hypertension index: novel prenatal predictor of severe postnatal pulmonary artery hypertension in antenatally diagnosed congenital diaphragmatic hernia

Purpose

This study aim to assess the potential of prenatal predictors of postnatal severe pulmonary artery hypertension (PAH) in isolated left congenital diaphragmatic hernia (CDH) and to define a new prenatal pulmonary hypertension index (PPHI).

Methods

A retrospective chart review of CDH patients between May 2005 and October 2008 was conducted. Ten patients with systemic/suprasystemic and 9 patients with subsystemic pulmonary hypertension at 3 weeks of age were identified. Diameters of the right pulmonary artery, left pulmonary artery (LPA_d), aorta, and the length of vermis of the cerebellum were obtained from prenatal magnetic resonance imaging to calculate the PPHI [=(LPA_d/length of vermis of the cerebellum) × 10] and the modified McGoon index (MGI) [=(diameter of the right pulmonary artery + LPA_d)/diameter of aorta]. Prenatal pulmonary hypertension index and MGI were compared with lung-to-head ratio, percent predicted lung volume, and total lung volume for pulmonary hypertension and survival.

Results

The PPHI and MGI had a significant, negative correlation with pulmonary hypertension (r = −0.61, P = .005, and r = −0.72, P < .005, respectively). The PPHI and MGI are significantly lower in the systemic/suprasystemic PAH group compared with the subsystemic PAH group (1.11 ± 0.32 versus 1.63 ± 0.28, P = .004, and 0.71 ± 0.15 versus 1.05 ± 0.11, P < .001, respectively). There were no significant differences between the groups comparing the lung-to-head ratio, percent predicted lung volume, and total lung volume.

Conclusion

Both PPHI and MGI accurately predict the severity of postnatal PAH in isolated left CDH.     

Systematic review and meta-analysis of the postnatal management of congenital cystic lung lesions

Background

Antenatally detected asymptomatic congenital cystic lung lesions may be managed conservatively or by surgical resection. We undertook a systematic review and meta-analysis to quantify the risks of elective surgery, emergency surgery, and observation.

Methods

All series published between 1996 and 2008, where the postnatal management of congenital cystic lung lesions was described, were reviewed. A meta-analysis was performed to determine whether elective or emergency surgery was associated with a higher risk of adverse outcomes.

Results

There were 41 reports describing 1070 patients (of whom 79% were antenatally detected). Five hundred five neonates survived without surgery into infancy, of whom only 16 (3.2%) became symptomatic. For all ages, elective surgery was associated with significantly less complications than emergency surgery. The risk ratio was 2.8 (95% confidence interval, 1.4-5.5; P < .005) when comparing complications after elective surgery with emergency surgery.

Conclusions

The risk of asymptomatic cases developing symptoms is small. However, elective surgery is associated with a better outcome than emergency surgery. If elective surgery is undertaken, it should be performed before 10 months. Although no prognostic indicators have so far been identified in the literature, a conservative approach may be appropriate for small lesions.