The Diagnostic Accuracy of Serum Alpha-Fetoprotein Levels in Follow-up for Recurrence of Sacrococcygeal Teratoma; a Nationwide Review of SCT Cases in the Netherlands.

BACKGROUND: Serum alpha-fetoprotein (AFP) is often used as tumour marker for recurrent sacrococcygeal teratoma (SCT). We aimed to assess the normal dynamics of serum AFP levels after initial resection and diagnostic accuracy of serum AFP levels the follow-up for recurrence in SCT. METHODS: This retrospective study included 57 patients treated for SCT in the six pediatric surgical centers in the Netherlands from 1980 to 2018. MAIN RESULTS: 57 patients were included in the study of whom 19 children developed 20 recurrences at a median of 14.0 months after initial resection. No significant difference was found in serum AFP level dynamics between the recurrence and non-recurrence group after initial resection (p = 0.950). Serum AFP levels did not significantly increase before recurrence (p = 0.106) compared to serum AFP levels of children without recurrence at the same time. However, serum AFP levels did significantly increase in malignant recurrences (n = 7) (p = 0.03) compared to patients without recurrence. A cut-off value of 55 µg/L was found to be predictive for recurrent SCT with an Area Under the Curve (AUC) of 0.636 with sensitivity of 50% and specificity of 100%. CONCLUSION: Dynamics of serum AFP levels are not different between patients with and without recurrence after initial resection of SCT. Serum AFP levels are not predictive for mature or immature recurrent SCT and normal AFP levels do not rule out recurrent SCT. However, serum AFP levels exceeding 55 µg/L can indicate recurrent SCT, especially malignant recurrences.

The Outcome of the Single Step Questionnaire in Pectus Excavatum Patients is Phase Dependent.

INTRODUCTION: Pectus excavatum (PE) is the most common chest wall deformity. Patients with PE may have cosmetic complaints, restricted physical capabilities, or both and may seek surgical correction. One method to assess satisfaction after surgery is the single step questionnaire (SSQ). Although the developers state that the SSQ produces a stabile score and only needs to be used once, we hypothesized that the score may depend on point in time after surgery. MATERIALS AND METHODS: One hundred and eight patients from a longitudinal cohort of patients undergoing a Nuss bar placement for PE were selected. Mean age was 16.0 years (range: 12-29). SSQ was completed at 6 weeks, 6 months, 1 year, and 2 years postoperatively. Mean and median scores per question and total scores were calculated on each measurement moment. Overall scores were tested using the Friedman test. RESULTS: There were significant differences in overall SSQ scores (p < 0.009) throughout the postoperative period, especially between 6 weeks and 6 months (p = 0.006). Scores on general health, exercise capacity, impact on social life, pain during hospital stay, and after discharge changed also significant in the first 2 years after Nuss bar placement. CONCLUSION: There were significant differences in total SSQ score depending on the time of application postoperatively. However, the most clinical relevant difference was between 6 weeks and 6 months. Assessment of the overall satisfaction postoperative with the SSQ questionnaire should not be done with a single measurement but rather at different postoperative time intervals before and after 6 months postoperatively.

Using three-dimensional ultrasound in predicting complex gastroschisis: A longitudinal, prospective, multicenter cohort study.

OBJECTIVE: To determine whether complex gastroschisis (ie, intestinal atresia, perforation, necrosis, or volvulus) can prenatally be distinguished from simple gastroschisis by fetal stomach volume and stomach-bladder distance, using three-dimensional (3D) ultrasound. METHODS: This multicenter prospective cohort study was conducted in the Netherlands between 2010 and 2015. Of seven university medical centers, we included the four centers that performed longitudinal 3D ultrasound measurements at a regular basis. We calculated stomach volumes (n = 223) using Sonography-based Automated Volume Count. The shortest stomach-bladder distance (n = 241) was determined using multiplanar visualization of the volume datasets. We used linear mixed modelling to evaluate the effect of gestational age and type of gastroschisis (simple or complex) on fetal stomach volume and stomach-bladder distance. RESULTS: We included 79 affected fetuses. Sixty-six (84%) had been assessed with 3D ultrasound at least once; 64 of these 66 were liveborn, nine (14%) had complex gastroschisis. With advancing gestational age, stomach volume significantly increased, and stomach-bladder distance decreased (both P < .001). The developmental changes did not differ significantly between fetuses with simple and complex gastroschisis, neither for fetal stomach volume (P = .85), nor for stomach bladder distance (P = .78). CONCLUSION: Fetal stomach volume and stomach-bladder distance, measured during pregnancy using 3D ultrasonography, do not predict complex gastroschisis.

Early cost-utility estimation of the surgical correction of pectus excavatum with the Nuss bar.

OBJECTIVES: The surgical correction of pectus excavatum (PE) with a Nuss bar provides satisfactory outcomes, but its cost-effectiveness is yet unproven. We prospectively analysed early outcomes and costs for Nuss bar placement. METHODS: Fifty-four patients aged 16 years or older (6 females and 48 males; mean age, 17.9 years; range 16.0-29.4 years) with a PE filled out a Short Form-36 Health Survey (SF-6D) preoperatively and 1 year after a Nuss procedure. Costs included professional fees and fees for the operating room, materials and hospital care. Changes in the responses to the SF-36 or its domains were compared using the Wilcoxon signed rank test and the utility test results were calculated preoperatively and postoperatively from the SF-6D. The quality-adjusted life years (QALYs) were calculated from the results of these tests. RESULTS: Significant improvements in physical functioning, social functioning, mental health and health transition (all P < 0.05) were noted. The other SF-36 subgroups showed improvement; however, the improvement was not significant. The SF-6D utility showed improvement from 0.76 preoperatively to 0.79 at the 1-year follow-up (P = 0.096). The mean direct costs were €8805. The 1-year discounted QALY gain was 0.03. The estimated cost-utility ratio was €293 500 per QALY gained. CONCLUSIONS: Despite a significant improvement in many domains of the SF-36, the results of the SF-6D cost-utility analysis showed only a small improvement in cost-effectiveness (> €80 000/QALY) for patients with PE 1 year after Nuss bar placement. Based on this discrepancy, general health outcome measurements as the basis for cost-utility analysis in patients with PE may not be the best way forward.

Sports activity in adolescents in the Netherlands with a pectus excavatum; the impact of surgery.

PURPOSE: Pectus excavatum (PE) is the most common chest wall deformity in adolescents. The main complaint is cosmetic, but many patients also complain about exertional dyspnea. This may lead to the patient seeking surgery of the thoracic wall deformity (TWD). The assumption is that both, appearance and physical complaints will have a negative effect on being able or wanting to engage in sport activity. METHODS: In December 2011 a prospective registration of sport activity in pectus excavatum patients started. Sport activity was assessed using questionnaires (CHQ, SF-36 and PEEQ). Measurements were taken before corrective surgery (preoperatively) and 12 months postoperatively. RESULTS: 127 patients have been included. The number of patients who were active in sports preoperatively and after 12 months remained steady. The type of sport activity, individual sport or team sport showed no significant change. The CHQ showed that physical activity caused fewer complaints (p < 0.001). The PEEQ showed a decrease in difficulties with sports activity performance after 12 months (p < 0.001). CONCLUSIONS: Twelve months after surgical correction of PE there was no significant increase in the number of patients performing sport activities. However there was a significant decrease of complaints or difficulties during sport compared to preoperatively. LEVEL OF EVIDENCE: Level IV.

The incidence of associated abnormalities in patients with sacrococcygeal teratoma.

BACKGROUND: Gross genetic causes for SCT are unknown; however, it might be associated with other abnormalities. We assessed the incidence of associated abnormalities in a large national cohort of neonates with SCT and aimed to identify predictive risk factors. PROCEDURE: The medical records were reviewed of 235 consecutive neonates with SCT treated at the six pediatric surgical centers in the Netherlands from 1970 to 2010. Potential risk factors for associated abnormalities analyzed included sex, gestational age, tumor-volume/histology and Altman-classification. RESULTS: In 76 patients (32.3%) at least one associated abnormality was diagnosed, with hydronephrosis as the most common (16.2%) and hip dysplasia in 4.3%. Multiple abnormalities were documented for 21 (9.0%). Prematurity and Altman type IV SCT were associated with an increased risk of any associated abnormality. No association between increased tumor-volume and hydronephrosis or hip dysplasia was found. Patients with type IV Altman SCT had a fourfold risk of suffering from hydronephrosis compared to Altman type I SCT. CONCLUSIONS: SCT was associated with other abnormalities in one-third of children. Some were tumor-related while others were related to prematurity or occurred sporadically. In contrast to clinically obvious anomalies, hip dysplasia or hydronephrosis might be latently present with more subtle clinical presentation. We therefore suggest renal- and hip-ultrasound in all patients, certainly those with Altman type IV SCT. LEVEL OF EVIDENCE RATING: Level II (retrospective study).

Outcome of isolated gastroschisis; an international study, systematic review and meta-analysis.

OBJECTIVE: To determine outcome of children born with isolated gastroschisis (no extra-gastrointestinal congenital abnormalities). STUDY DESIGN: International cohort study and meta-analysis. PRIMARY OUTCOME: time to full enteral feeding (TFEF); secondary outcomes: Duration of mechanical ventilation, length of stay (LOS), mortality and differences in outcome between simple and complex gastroschisis (complex; born with bowel atresia, volvulus, perforation or necrosis). To compare the cohort study results with literature three databases were searched. Studies were eligible for inclusion if cases were born in developed countries with isolated gastroschisis after 1990, number of cases >20 and TFEF was reported. RESULTS: The cohort study included 204 liveborn cases of isolated gastroschisis. The TFEF, median duration of ventilation and LOS was, 26days (range 6-515), 2days (range 0-90) and 33days (range 11-515), respectively. Overall mortality was 10.8%. TFEF and LOS were significantly longer (P<0.0001) and mortality was fourfold higher in the complex group. Seventeen studies, amongst the current study, were included for further meta-analysis comprising a total of 1652 patients. Mean TFEF was 35.3±4.4days, length of ventilation was 5.5±2.0days, LOS was 46.4±5.2days and mortality risk was 0.06 [0.04-0.07 95%CI]. Outcome of simple and complex gastroschisis was described in five studies. TFEF, ventilation time, LOS were significant longer and mortality rate was 3.64 [1.95-6.83 95%CI] times higher in complex cases. CONCLUSIONS: These results give a good indication of the expected TFEF, ventilation time and LOS and mortality risk in children born with isolated gastroschisis, although ranges remain wide. This study shows the importance of dividing gastroschisis into simple and complex for the prediction of outcome.

Hemorrhage is the most common cause of neonatal mortality in patients with sacrococcygeal teratoma.

BACKGROUND: A small percentage of neonates with sacrococcygeal teratoma die shortly after birth from hemorrhagic complications. The incidence of and risk factors associated with hemorrhagic mortality are unknown. In this multicenter study we determined the incidence of early death in neonates born with SCT and evaluated potential risk factors for hemorrhagic mortality. METHODS: 235 children with SCT treated from 1970 to 2010 in the Netherlands were retrospectively included. The following candidate risk factors for hemorrhagic mortality were examined: sex, prematurity, Altman type, tumor volume, tumor histology, necessity of emergency operation and time of diagnosis. RESULTS: Eighteen patients (7.7%) died at a median age of 163.5days (range 1.7-973days). Nine patients died of a malignancy. Nine others (3.8%) died postnatally (age 1-27days), six even within two days after birth. In seven of these nine patients death was related to tumor-hemorrhage and/or circulatory failure. Risk factors for hemorrhagic mortality were prematurity, tumor volume>1000cm3 and performance of an emergency operation. CONCLUSIONS: Hemorrhagic mortality of neonates with SCT is relatively high (3.8%) representing almost 70% of the overall mortality in the neonatal period. High-output cardiac failure, internal tumor hemorrhage and perioperative bleeding were the most common causes of early death and were all strongly associated with larger tumor sizes. LEVEL-OF-EVIDENCE RATING: II (Retrospective study).

Surgical management and autologous intestinal reconstruction in short bowel syndrome.

Short bowel syndrome (SBS) is a serious condition with considerable morbidity and mortality. When treatment with parenteral nutrition fails and life-threatening complications occur, autologous intestinal reconstruction (AIR) should be considered before intestinal transplantation (ITx). Single or combined ITx should be reserved for patients with severe liver disease and as last resort in the treatment of SBS. Longitudinal intestinal lengthening and tailoring (LILT) has proven its value in AIR, but its availability depends on the expertise of the surgeons. Serial transverse enteroplasty (STEP) has similar success rates as LILT and fewer patients progress to ITx. STEP is also applicable at small bowel dilatation in ultra-short bowel syndrome. The scope may be widened when duodenal dilatation can be treated as well. Spiral intestinal lengthening and tailoring (SILT) is a promising alternative. More research is needed to confirm these findings. Therefore we suggest an international data registry for all intestinal lengthening procedures.

Patient-Reported Defecation and Micturition Problems Among Adults Treated for Sacrococcygeal Teratoma During Childhood--The Need for New Surveillance Strategies.

BACKGROUND: To evaluate defecation and micturition complaints in adults treated for sacrococcygeal teratoma (SCT) during childhood and to identify risk factors for soiling, urinary incontinence, and constipation beyond childhood. PROCEDURE: Records of patients aged ≥18 treated for SCT during infancy in the Netherlands were retrospectively reviewed. Frequency and severity of soiling, constipation, and urinary incontinence were evaluated using questionnaires designed in line with the Krickenbeck classification. Problems during childhood were compared to outcomes at adult age in part of the cohort. Associations between patient- and disease-related factors with complaints beyond childhood were analyzed with the chi-square test or Fisher's exact test, when appropriate. RESULTS: Of 47 included patients (mean age 26.2 years, SD ±6.5), 49% reported at least one defecation or micturition complaint. Urinary incontinence was present in 30% and had a greater negative impact than soiling (24%). Ten patients (21%) reported constipation; five found this severely bothering. Three patients reported social restrictions due to defecation or micturition complaints (6.4%). While sex and tumor histology were not identified as risk factors, a tumor diameter of >10 cm and Altman type I or type II SCT were associated with constipation during adulthood. CONCLUSIONS: One-third of the patients treated for SCT during childhood reported urinary and defecation problems beyond childhood. In only a minority of cases, these led to social restrictions. A greater tumor diameter was associated with a higher risk of constipation during adulthood. Prolonged surveillance strategies are advised for all patients with SCT.

Sexual function after treatment for sacrococcygeal teratoma during childhood.

BACKGROUND: Children treated for sacrococcygeal teratoma (SCT) may suffer from sexual dysfunction later in life because of the extended pelvic surgery performed, however, structured evaluations have not been performed yet. METHODS: The Female Sexual Function Index (FSFI), the International Index of Erectile Function (IIEF) and the Body Image Questionnaire (BIQ) were sent to patients (≥18years) treated for SCT in the Dutch pediatric surgical centers after 1970. RESULTS: Forty-five of 76 patients returned the questionnaires; 28 women (median age 27.3years, range 18.3-41.0) and seven men (median age 22.0years, range 19.1-36.5) were eligible for analysis. The FSFI and IIEF results were compared to healthy controls. Female patients scored significantly lower on the desire (p=0.014), arousal (p=0.013) and lubrication domain (p=0.019). FSFI total-scores of female patients were significantly lower compared to controls [median 30.5 (IQR 28.6-31.4) vs. median 32.4 (IQR 30.6-33.45) p≤0.001] but were above the threshold value for sexual dysfunction. Males reported normal erectile function and penetration ability with normal ejaculation. Females had significant lower BIQ results compared to males; BIQ-cosmesis scores were moderately correlated to the FSFI-desire score (r=-0.37, p=0.028). CONCLUSION: SCT resection in girls may result in diminished sexual function at adult age with worse self-perceived body image. The possibility of sexual complaints should be integrated in the surveillance strategies for these patients.

Evaluation of chemotherapeutic sequelae and quality of life in survivors of malignant sacrococcygeal teratoma.

PURPOSE: The impact of chemotherapeutic sequelae on long-term quality of life (QoL) for survivors of malignant sacrococcygeal teratoma (SCT) is unknown. The incidence of chemotherapeutic toxicity in patients treated for malignant SCT and possible effects on the QoL were analyzed. METHODS: Retrospective chart review of patients ≥18 years treated for SCT in the Netherlands was performed. Present QoL was evaluated using the SF-36 questionnaire. The results of survivors of malignant SCT were compared to those of patients treated for benign SCT. RESULTS: Fifty-one of 76 traceable patients consented to participate. The results of 47 (92.2 %), 9 men and 38 women (median age 25.4 years, range 18.3-41.2), were analyzed. Eleven had been treated for malignancy; 63.6 % suffered from at least one chemotherapeutic sequel with hearing loss as the most common one. Results for both groups were similar on all but one SF-36 subcategory; those treated for malignant tumor scored significantly lower on the subcategory physical functioning (p = 0.02). CONCLUSION: Despite the high incidence of chemotherapeutic sequelae among survivors of malignant SCT, their QoL does not differ from that of those treated for benign SCT. Even though their physical functioning is restricted, daily activities and psychosocial functioning of survivors of malignant SCT are not restricted.

[A neonate with aganglionosis of the entire colon]. / Een neonaat met aganglionose van het gehele colon.

BACKGROUND: 1 in every 5,000 children is born with Hirschsprung's disease (HD). Total colonic aganglionosis is an extended form of HD and is present in 2-13% of all patients with the disease. CASE DESCRIPTION: Paediatricians from a general hospital referred a 12-day-old male neonate on account of suspected abdominal obstruction and sepsis. An ileostomy was created and biopsies were taken during a laparotomy. Examination of the biopsies indicated total colonic aganglionosis. This diagnosis was unexpected because the symptoms of obstruction had occurred late and the radiological findings were not characteristic of Hirschsprung's disease. CONCLUSION: Persistent passage problems after delayed meconium passage are important alarm symptoms of Hirschsprung's disease. This diagnosis must be considered and ruled out in these patients in order to avoid an acute presentation of this disease.

Malignant transformation in sacrococcygeal teratoma and in presacral teratoma associated with Currarino syndrome: a comparative study.

BACKGROUND/PURPOSE: The risk of malignant transformation of sacrococcygeal teratoma (SCT) and of presacral teratoma in Currarino syndrome (CS) may differ despite the similar position and appearance. METHODS: Malignant transformation and teratoma recurrence were assessed in a national retrospective comparative analysis of 205 SCT and 16 CS patients treated in one of the six pediatric surgical centers in the Netherlands between January 1981 and December 2010. RESULTS: The malignancy free survival of patients with SCT was lower than for patients with a presacral teratoma associated with CS (80% and 58% after one and two years in SCT versus 100% after two years in CS, p=0.017) CONCLUSIONS: In SCT, malignancy and recurrence risk are high. Therefore, early and complete resection is mandatory. Our data show that the risk of malignant transformation of a presacral teratoma in CS is small.

Quality of life in adulthood after resection of a sacrococcygeal teratoma in childhood: a Dutch multicentre study.

OBJECTIVE: Children treated for sacrococcygeal teratoma (SCT) may experience functional sequelae later in life. It is not known whether SCT and associated problems affect the patient's general quality of life (QoL). In a national survey, we evaluated general QoL in adults treated for SCT during childhood and compared the results to reference values for the Dutch population. DESIGN: The records of patients aged ≥18 years treated for an SCT in one of the six paediatric surgical centres in the Netherlands from 1970 to 1993 were retrospectively reviewed; patient characteristics were retrieved from medical records. General QoL was evaluated using the Short Form 36 Health Survey (SF-36). The means of the eight SF-36 domain scores of patients treated for SCT were compared to reference values for the Dutch population (n=757, aged 18-43 years). Linear regression analysis was used to adjust for differences in baseline characteristics between both groups. RESULTS: 46 of 51 patients treated for SCT during childhood (90.2%), with a mean age of 26.3 years (range 18.3-41.1), returned completed SF-36 questionnaires. Their scores on all SF-36 subcategories were equivalent to those of the Dutch reference population. No significant differences in the scores of the SF-36 subcategories were found after linear regression analysis adjusting for differences in age, sex and living status between both groups. CONCLUSIONS: The long-term QoL of patients treated for SCT during childhood does not differ from that of the general population. Moreover, patients do not show impairment in social, physical or emotional functioning in adulthood.

Evaluation of pregnancy and delivery in 13 women who underwent resection of a sacrococcygeal teratoma during early childhood.

BACKGROUND: Sacrococcygeal teratoma resection often brings changes in pelvic anatomy and physiology with possible consequences for defecation, micturition and sexual function. It is unknown, whether these changes have any gynecological and obstetric sequelae. Until now four pregnancies after sacrococcygeal teratoma resection have been described and cesarean section has been suggested to be the method of choice for delivery. We evaluated the pregnancy course and mode of delivery in women previously treated for a sacrococcygeal teratoma. METHODS: The records of all patients who underwent sacrococcygeal teratoma resection after 1970 in one of the six pediatric surgical centers in the Netherlands were reviewed retrospectively. Women aged 18 years and older were eligible for participation. Patient characteristics, details about the performed operation and tumor histology were retrieved from the records. Consenting participants completed a questionnaire addressing fertility, pregnancy and delivery details. RESULTS: Eighty-nine women were eligible for participation; 20 could not be traced. Informed consent was received from 41, of whom 38 returned the completed questionnaire (92.7%). Thirteen of these 38 women conceived, all but one spontaneously. In total 20 infants were born, 17 by vaginal delivery and 3 by cesarean section, in one necessitated by previous intra-abdominal surgery as a consequence of sacrococcygeal teratoma resection. Conversion to a cesarean section was never necessary. None of the 25 women without offspring reported involuntary childlessness. CONCLUSIONS: There are no indications that resection of a sacrococcygeal teratoma in female patients is associated with reduced fertility: spontaneous pregnancy is possible and vaginal delivery is safe for mother and child, irrespective of the sacrococcygeal teratoma classification or tumor histology.

[Infestation with Enterobius vermicularis mimicking appendicitis]. / Appendicitis-beeld door Enterobius vermicularis.

BACKGROUND: Gastrointestinal infestation with the parasite Enterobius vermicularis is common in humans and is usually harmless. Anal pruritus is the most characteristic symptom, but the parasites can cause severe abdominal pain mimicking appendicitis. Early recognition can prevent an unnecessary appendectomy. CASE DESCRIPTION: A six-year-old girl reported to the accident and emergency department with pain in the lower right abdominal region. She was admitted and treated for suspected perforated appendix, following physical examination supplemented with an abdominal CT scan. After antibiotic treatment the symptoms disappeared as did the abscess, apart from a minor amount of residual infiltrate. She was then readmitted twice with recurrent abdominal pain without radiological evidence of an abdominal focus. We decided to conduct a diagnostic laparoscopy and an elective appendectomy à froid. During this procedure living worms were found in the appendix. Treatment with the anthelminthicum mebendazol was effective. CONCLUSION: Gastro-intestinal infestation with E. vermicularis is very common, especially in young children. This infestation is usually harmless, but can mimic appendicitis. This infestation is easily treatable with mebendazol.

Acute small bowel volvulus in a 2-month-old infant.

KEY CLINICAL MESSAGE: Abdominal cystic lymphangiomas are rare and occur secondary to congenital malformation of the lymphatics, mostly in the mesenterium. Acute or chronic volvulus of the small bowel may occur by traction of the lymphangioma. Therapy includes resection of the lymphangioma and of the small bowel involved.

Diagnosis and treatment of lymph node metastases in pediatric rhabdomyosarcoma in the Netherlands: a retrospective analysis.

BACKGROUND: In pediatric rhabdomyosarcoma (RMS), evaluation of lymph node involvement (N1) is an important staging aspect, but difficult to assess. The aim of our study was to evaluate the assessment of lymph node infiltration and impact on outcome in N1 RMS patients. METHODS: We identified 277 non-metastatic RMS patients diagnosed and treated between 1990 and 2008. Patients with recorded N1 disease were evaluated for their diagnostic procedures and outcome. RESULTS: In 13.7% N1 status was reported. In 19 of 34 N1 patients, lymph node biopsies were performed for histologically confirmation. Different treatment modalities were used to treat lymph node metastases. In total 23 of 31 patients received local treatment of the node (11/23 RT, 4/23 surgery, and 8/23 both). All patients received chemotherapy. Lymph node relapse occurred in 7 of 31 patients who were treated with one or two modalities. Only 1 (14%) of 8 patients treated with three modalities relapsed. In N0 patients 10 (4.2%) of 239 had a regional lymph node relapse, and 9 of 10 died. CONCLUSION: Lymph node metastases are an essential part of staging. Node positivity contributes to relapse of disease. Nodal relapse is also associated with a high mortality rate. These data imply that nodal assessment needs to be optimal and standardized for improved staging.

Outcome of infants presenting with echogenic bowel in the second trimester of pregnancy.

OBJECTIVE: Fetal echogenic bowel (FEB) is a soft marker found on second trimester sonography. Our main aim was to determine the outcome of infants who presented with FEB and secondarily to identify additional sonographic findings that might have clinical relevance for the prognosis. DESIGN: We reviewed all pregnancies in which the diagnosis FEB was made in our Fetal Medicine Unit during 2009-2010 (N=121). We divided all cases into five groups according to additional sonographic findings. Group 1 consisted of cases of isolated FEB, group 2 of FEB associated with dilated bowels, group 3 of FEB with one or two other soft markers, group 4 of FEB with major congenital anomalies or three or more other soft markers, and group 5 consisted of FEB with isolated intrauterine growth restriction (IUGR). RESULTS: Of 121 cases, five were lost to follow-up. Of the remaining 116 cases, 48 (41.4%) were assigned to group 1, 15 (12.9%) to group 2, 15 (12.9%) to group 3, 27 (23.2%) to group 4, and 11 (9.5%) to group 5. The outcome for group 1 was uneventful. In group 2 and 3, two anomalies, anorectal malformation and cystic fibrosis, were detected postnatally (6.7%). In group 4, mortality and morbidity were high (78% resp. 22%). Group 5 also had high mortality (82%) and major morbidity (18%). CONCLUSIONS: If FEB occurs in isolation, it is a benign condition carrying a favourable prognosis. If multiple additional anomalies or early IUGR are observed, the prognosis tends to be less favourable to extremely poor.