Omphalocele and biliary atresia: chance or causality. A case report.

To relate omphalocele and biliary atresia and investigate possible embryological correlations that justify the simultaneous occurrence. A female preterm newborn diagnosed as omphalocele; cesarean delivery, weight 2,500g, 46 XX karyotype. Initially, the newborn remained fasting and on parenteral nutrition, and enteral diet was introduced later, with good acceptance. On the 12th day of life, the newborn presented direct hyperbilirubinemia, increased levels of liver enzymes and fecal acholia, with a presumptive diagnosis of biliary atresia. However, the ultrasound was inconclusive, due to anatomical changes resulting from omphalocele. A surgical approach was chosen on the 37th day of life aiming to confirm diagnosis of biliary atresia and to repair omphalocele. During the surgical procedure, structural alterations compatible with biliary atresia were observed, later confirmed by pathological examination; a hepatoportoenterostomy was performed and the omphalocele was corrected. She evolved well in the postoperative period, with a decrease in direct bilirubin and liver enzymes, as well as resolution of fecal acholia, and was discharged in good clinical condition. This is a bizarre and extremely rare association, but the prognosis may be good when an early diagnosis is made and surgery performed, besides support and clinical management to prevent complications in the perioperative period. Although the pathogenesis of the diseases has not been fully defined yet, there is, to date, no direct relation between them. The association between omphalocele and biliary atresia is extremely uncommon, with only two published cases.

Omphalocele and biliary atresia: chance or causality. A case report

ABSTRACT To relate omphalocele and biliary atresia and investigate possible embryological correlations that justify the simultaneous occurrence. A female preterm newborn diagnosed as omphalocele; cesarean delivery, weight 2,500g, 46 XX karyotype. Initially, the newborn remained fasting and on parenteral nutrition, and enteral diet was introduced later, with good acceptance. On the 12th day of life, the newborn presented direct hyperbilirubinemia, increased levels of liver enzymes and fecal acholia, with a presumptive diagnosis of biliary atresia. However, the ultrasound was inconclusive, due to anatomical changes resulting from omphalocele. A surgical approach was chosen on the 37th day of life aiming to confirm diagnosis of biliary atresia and to repair omphalocele. During the surgical procedure, structural alterations compatible with biliary atresia were observed, later confirmed by pathological examination; a hepatoportoenterostomy was performed and the omphalocele was corrected. She evolved well in the postoperative period, with a decrease in direct bilirubin and liver enzymes, as well as resolution of fecal acholia, and was discharged in good clinical condition. This is a bizarre and extremely rare association, but the prognosis may be good when an early diagnosis is made and surgery performed, besides support and clinical management to prevent complications in the perioperative period. Although the pathogenesis of the diseases has not been fully defined yet, there is, to date, no direct relation between them. The association between omphalocele and biliary atresia is extremely uncommon, with only two published cases.

Current management of biliary atresia based on 35 years of experience at a single center.

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

Current management of biliary atresia based on 35 years of experience at a single center

OBJECTIVE: The prognosis of patients with biliary atresia undergoing Kasai portoenterostomy is related to the timing of the diagnosis and the indication for the procedure. The purpose of the present study is to present a practical flowchart based on 257 children who underwent Kasai portoenterostomy. METHODS: We conducted a retrospective cohort study of patients who underwent Kasai portoenterostomy between 1981 and 2016. RESULTS: During the first period (1981 to 2009), 230 infants were treated, and the median age at the time of surgery was 84 days; jaundice was resolved in 77 patients (33.5%). During the second period, from 2010 to 2016, a new diagnostic approach was adopted to shorten the wait time for portoenterostomy; an ultrasonography examination suggestive of the disease was followed by primary surgical exploration of the biliary tract without complementary examination or liver biopsy. Once the diagnosis of biliary atresia was confirmed, a portoenterostomy was performed during the same surgery. During this period, 27 infants underwent operations; the median age at the time of surgery was 66 days (p<0.001), and jaundice was resolved in 15 patients (55.6% - p=0.021), with a survival rate of the native liver of 66.7%. CONCLUSION: Primary surgical exploration of the biliary tract without previous biopsy was effective at improving the prognostic indicators of patients with biliary atresia undergoing Kasai portoenterostomy.

Prematurity and fetal lung response after tracheal occlusion in fetuses with severe congenital diaphragmatic hernia.

OBJECTIVE: To evaluate the independent association of fetal pulmonary response and prematurity to postnatal outcomes after fetal tracheal occlusion for congenital diaphragmatic hernia. METHODS: Fetal pulmonary response, prematurity (<37 weeks at delivery) and extreme prematurity (<32 weeks at delivery) were evaluated and compared between survivors and non-survivors at 6 months of life. Multivariable analysis was conducted with generalized linear mixed models for variables significantly associated with survival in univariate analysis. RESULTS: Eighty-four infants were included, of whom 40 survived (47.6%) and 44 died (52.4%). Univariate analysis demonstrated that survival was associated with greater lung response (p=0.006), and the absence of extreme preterm delivery (p=0.044). In multivariable analysis, greater pulmonary response after FETO was an independent predictor of survival (aOR 1.87, 95% CI 1.08-3.33, p=0.023), whereas the presence of extreme prematurity was not statistically associated with mortality after controlling for fetal pulmonary response (aOR 0.52, 95% CI 0.12-2.30, p=0.367). CONCLUSION: Fetal pulmonary response after FETO is the most important factor associated with survival, independently from the gestational age at delivery.

Living donor liver transplantation in children: should the adult donor be operated on by an adult or pediatric surgeon? Experience of a single pediatric center.

BACKGROUND/PURPOSE: Living donor liver transplantation has become a cornerstone for the treatment of children with end-stage hepatic dysfunction, especially within populations or countries with low rates of organ utilization from deceased donors. The objective is to report our experience with 185 living donors operated on by a team pediatric surgeons in a tertiary center for pediatric liver transplantation. METHODS: Retrospective analysis of medical records of donors of hepatic grafts for transplant undergoing surgery between June 1998 and March 2013. RESULTS: Over the last 14 years, 185 liver transplants were performed in pediatric recipients of grafts from living donors. Among the donors, 166 left lateral segments (89.7%), 18 left lobes without the caudate lobe (9.7%) and 1 right lobe (0.5%) were harvested. The donor age ranged from 16 to 53 years, and the weight ranged from 47 to 106 kg. In 10 donors, an additional graft of the donor inferior mesenteric vein was harvested to substitute for a hypoplastic recipient portal vein. The transfusion of blood products was required in 15 donors (8.1%). The mean hospital stay was 5 days. No deaths occurred, but complications were identified in 23 patients (12.4%): 9 patients experienced abdominal pain and severe gastrointestinal symptoms and 3 patients required reoperations. Eight donors presented with minor bile leaks that were treated conservatively, and 3 patients developed extra-peritoneal infections (1 wound collection, 1 phlebitis and 1 pneumonia). Eight grafts (4.3%) showed primary dysfunction resulting in recipient death (3 cases of fulminant hepatitis, 1 patient with metabolic disease, 1 patient with Alagille syndrome and 3 cases of biliary atresia in infants under 1 year old). There was no relation between donor complications and primary graft dysfunction (P=0.6). CONCLUSIONS: Living donor transplantation is safe for the donor and presents a low morbidity. The donor surgery may be performed by a team of trained pediatric surgeons.

Quantitative lung index, contralateral lung area, or lung-to-head ratio to predict the neonatal outcome in isolated congenital diaphragmatic hernia?

OBJECTIVES: To estimate the accuracy of the quantitative lung index and contralateral lung area for prediction of the neonatal outcome in isolated congenital diaphragmatic hernia in comparison to other available prediction models. METHODS: Between January 2004 and December 2010, 108 fetuses with isolated (82 left-sided and 26 right-sided) congenital diaphragmatic hernia were prospectively evaluated. The quantitative lung index and observed-to-expected contralateral lung area were measured and compared to the neonatal survival rate and severe postnatal pulmonary arterial hypertension, along with the lung-to-head ratio, observed-to-expected lung-to-head ratio, and observed-to-expected total lung volume. RESULTS: Overall neonatal mortality was 64.8% (70 of 108). Severe pulmonary arterial hypertension was diagnosed in 68 (63.0%) of the cases, which was associated with neonatal death (P < .001). Both the quantitative lung index and observed-to-expected contralateral lung area were significantly associated with neonatal survival and pulmonary arterial hypertension (P < .001), with accuracy to predict survival of 70.9% and 70.0%, respectively, and accuracy to predict hypertension of 78.7% and 72.0%; however, they were both less accurate than the observed-to-expected total lung volume (83.3% and 86.1%; P < .01). The lung-to-head ratio (73.1% and 78.7%) and observed-to-expected lung-to-head ratio (75.9% and 72.2%; P > .05) had similar accuracy as the quantitative lung index and observed-to-expected contralateral lung area. CONCLUSIONS: The observed-to-expected total lung volume is the most accurate predictor of the neonatal outcome in cases of isolated congenital diaphragmatic hernia. Both the quantitative lung index and observed-to-expected contralateral lung area, albeit reasonably accurate, do not produce the same level of accuracy and render similar results as the lung-to-head ratio and observed-to-expected lung-to-head ratio.

Percutaneous laser ablation under ultrasound guidance for fetal hyperechogenic microcystic lung lesions with hydrops: a single center cohort and a literature review.

OBJECTIVE: To evaluate the perinatal outcomes in hydropic fetuses with congenital microcystic pulmonary lesions that underwent percutaneous, invasive, laser therapy. METHOD: This retrospective study reviews the literature and our experience between 2004 and 2010. Characteristics of the cystic lung lesions, liquor volume (presence of polyhydramnios or not), localization of ablation (vascular vs interstitial) and gestational age at which the procedure was performed were related to outcome (survival). RESULTS: In total, 16 fetuses with congenital lung lesions underwent 'invasive' percutaneous laser ablation, seven performed in our center and nine published cases. Survival rate was higher in fetuses with a subsequent postnatal diagnosis of bronchopulmonary sequestration (87.5%) compared with congenital adenomatoid malformation (28.6%; p = 0.04). The technique of vascular ablation was more successful (100%) than interstitial ablation (25.0%, p < 0.01). CONCLUSION: Percutaneous vascular laser ablation seems to be effective for bronchopulmonary sequestration in hydropic fetuses. Outcomes were worst following interstitial ablation for microcystic congenital adenomatoid with hydrops.

Fetal pulmonary response after fetoscopic tracheal occlusion for severe isolated congenital diaphragmatic hernia.

OBJECTIVE: To estimate the response in lung growth and vascularity after fetal endoscopic tracheal occlusion for severe congenital diaphragmatic hernia in the prediction of neonatal survival. METHODS: Between January 2006 and December 2010, fetal lung parameters (observed-to-expected lung-to-head ratio; observed-to-expected lung volume; and contralateral lung vascularization index) were evaluated before fetal tracheal occlusion and were evaluated longitudinally every 2 weeks in 72 fetuses with severe isolated congenital diaphragmatic hernia. Thirty-five fetuses underwent fetal endoscopic tracheal occlusion and 37 cases did not. RESULTS: Survival rate was significantly higher in the fetal endoscopic tracheal occlusion group (54.3%) than in the no fetal endoscopic tracheal occlusion group (5.4%, P<.01). Fetal endoscopic tracheal occlusion resulted in a significant improvement in fetal lung size and pulmonary vascularity when compared with fetuses that did not go to the fetal intervention (increase of the observed-to-expected lung-to-head ratio, observed-to-expected total lung volume, and contralateral pulmonary vascularization index 56.2% compared with 0.3%, 37.9% compared with 0.1%, and 98.6% compared with 0.0%, respectively; P<.01). Receiver operating characteristic curves indicated that the observed-to-expected total fetal lung volume was the single best predictor of neonatal survival before fetal endoscopic tracheal occlusion (cutoff 0.23, area under the curve [AUC] 0.88, relative risk 5.3, 95% confidence interval [CI] 1.4-19.7). However, the contralateral lung vascularization index at 4 weeks after fetal endoscopic tracheal occlusion was more accurate in the prediction of neonatal outcome (cutoff 24.0%, AUC 0.98, relative risk 9.9, 95% CI 1.5-66.9) with the combination of observed-to-expected lung volumes and contralateral lung vascularization index at 4 weeks being the best predictor of outcome (AUC 0.98, relative risk 16.6, 95% CI 2.5-112.3). CONCLUSION: Fetal endoscopic tracheal occlusion improves survival rate by increasing the lung size and pulmonary vascularity in fetuses with severe congenital diaphragmatic hernia. The pulmonary response after fetal endoscopic tracheal occlusion can be used to predict neonatal survival.

[Conjoined twins: prenatal diagnosis, delivery and postnatal outcome]. / Gêmeos unidos: diagnóstico pré-natal, parto e desfecho após o nascimento.

PURPOSE: The aim of this study was to analyze conjoined twins in terms of antenatal, delivery and postnatal aspects. METHODS: A retrospective descriptive analysis of prenatally diagnosed conjoined twins. Prenatal ultrasound and echocardiography, delivery details, postnatal follow-up, surgical separation and post mortem data were reviewed. The twins were classified according to the type of fusion between fetal structures. The following data were analyzed: ultrasound and echocardiographic findings, antenatal lethality and possibility of surgical separation, delivery details and survival rates. RESULTS: Forty cases of conjoined twins were included in the study. There were 72.5% cases of thoracophagus, 12.5% of paraphagus, 7.5% of omphalo-ischiophagus, 5.0% of omphalophagus, and 2.5% of cephalophagus. Judicial termination of pregnancy was requested in 58.8% of the cases. Cesarean section was performed in all cases in which pregnancy was not terminated. The mean gestational age at delivery was 35 weeks; all twins were live births with a mean birth weight of 3,860 g and 88% died postnatally. Ten percent of the live borns were submitted to surgical separation with a 60% survival rate. The total survival rate was 7.5% and postnatal survival was 12%. Antenatal evaluation of lethality and possibility of surgical separation were precise. There were no maternal complications related to delivery. CONCLUSION: Conjoined twins present a dismal prognosis mainly related to the complex cardiac fusion present in the majority of cases with thoracic sharing. At referring centers, prenatal ultrasound and echocardiographic evaluation accurately delineate fetal prognosis and the possibility of postnatal surgical separation.

Comparison between fetal endoscopic tracheal occlusion using a 1.0-mm fetoscope and prenatal expectant management in severe congenital diaphragmatic hernia.

OBJECTIVES: To evaluate if fetal endoscopic tracheal occlusion (FETO) for severe congenital diaphragmatic hernia (CDH) using a 1.0-mm fetoscope improves neonatal outcome. METHOD: Between January 2006 and December 2008, a controlled study was conducted at a single center in which FETO was proposed for fetuses with severe isolated CDH (lung-to-head ratio <1.0) and liver herniation to the thoracic cavity but no other detectable anomalies at diagnosis (<26 weeks). FETO was performed under maternal epidural and fetal intramuscular anesthesia, guided by ultrasonography and 1.0-mm fetoscope between 26 and 30 weeks. All cases submitted to FETO were delivered by ex utero intrapartum therapy procedure. Postnatal therapy was the same for both treated fetuses and controls. The primary outcome was neonatal survival (up to 28 days after birth). RESULTS: A total of 35 women met the inclusion criteria, and in 17 of them, fetal intervention was intended. However, in 1 case, it was not possible to insert the balloon inside the fetal trachea because of placental bleeding. FETO was therefore successfully performed in 16 fetuses with severe CDH. Eighteen cases received no prenatal intervention and served as the control group. Mean gestational age at diagnosis was similar in both groups (p > 0.05). Delivery occurred at 35.6 (range: 28-38) weeks in the FETO group and at 37.5 (range: 31-40) weeks (p = 0.18) among controls. Nine of 17 (52.9%) infants in the FETO group and 1 of 18 (5.6%) in the control group survived (p < 0.01). Severe pulmonary arterial hypertension was present in 8/17 (47.1%) infants from the FETO group and in 16/18 (88.9%) controls (p = 0.01). CONCLUSION: The present study shows that FETO using a 1.0-mm fetoscope is feasible and may improve neonatal outcome in severe CDH.

Congenital intrahepatic arterioportal fistula presenting as severe undernutrition and chronic watery diarrhea in a 2-year-old girl.

Intrahepatic arterioportal fistula (IAPF) is a rare cause of portal hypertension in young children. We report the case of a 2-year-old girl with severe undernutrition, chronic watery diarrhea, and gastrointestinal bleeding because of a congenital intrahepatic arterioportal fistula. Radiographic embolization and surgical ligation of the left hepatic artery were attempted, with no resolution of the symptoms. So, a left lobectomy was performed, with excellent results and prompt disappearance of the diarrhea. Hepatectomy should be considered as a definitive and reliable therapy for congenital IAPF.

Intimal dissection of the hepatic artery after thrombectomy as a cause of graft loss in pediatric living-related liver transplantation.

HAT is the main cause of graft loss in pediatric living-related LTx. Revascularization of the graft by thrombectomy and re-anastomosis has been reported to be effective for graft salvage in cases of HAT and should be attempted when potential donors are not available for emergency re-transplantation. Immediate complications secondary to revascularization attempts in cases of HAT are not described. Late complications are mainly related to biliary tree ischemia. We report a case of child who experienced intimal hepatic artery dissection, which extended into intra-hepatic branches of the artery after a thrombectomy with a Fogarty balloon catheter in an attempt to restore arterial flow after HAT. This complication led to acute deterioration of the graft and the need for emergency re-transplantation.

Prenatal diagnosis and perinatal outcome of 38 cases with congenital diaphragmatic hernia: 8-year experience of a tertiary Brazilian center.

PURPOSE: To evaluate the perinatal results for neonates with congenital diaphragmatic hernia diagnosed prenatally. METHOD: We reviewed data from 38 cases of congenital diaphragmatic hernia diagnosed prenatally from January 1995 to December 2003 in the Fetal Medicine Unit of the Department of Obstetrics and Gynecology, São Paulo University Medical School. The main data analyzed were gestational age at diagnosis, fetal karyotyping, side of diaphragmatic defect, presence of associated structural malformations, hepatic herniation, and severe mediastinal shift. Perinatal outcomes were obtained by reviewing hospital documents or by directly calling the patients' immediate relatives. RESULTS: Mean gestational age at diagnosis was 29 weeks (range, 16-37 weeks). Thirty (79%) cases had a left diaphragmatic defect and 8 (21%) had a right lesion. Associated structural malformations were observed in 21 (55%) cases, in which 12 fetuses had a normal karyotype and 9 had chromosomal abnormalities. Isolated congenital diaphragmatic hernia was confirmed in 17 (45%) cases. The overall perinatal mortality rate was 92%. Rates of fetal deaths, early neonatal deaths, late neonatal deaths, and survival were 42%, 50%, 0%, and 8%, respectively, in cases with associated structural malformations but normal karyotyping; 56%, 44%, 0%, and 0% for cases with chromosomal abnormalities; and, 0%, 76%, 12%, and 12% in cases with isolated congenital diaphragmatic hernia. The neonatal mortality rate was 89% in cases with isolated congenital diaphragmatic hernia. CONCLUSION: Perinatal mortality was very high in prenatally diagnosed cases of congenital diaphragmatic hernia. Earlier perinatal deaths are associated with the presence of other structural defects or chromosomal abnormalities. In cases of isolated congenital diaphragmatic hernia, mortality is related to the presence of herniated liver, right-sided lesion, and major mediastinal shift.

The outcome of newborns with abdominal wall defects according to the method of abdominal closure: the experience of a single center.

Recent reports suggest that the technique of abdominal closure in neonates with anterior abdominal wall defects (AWD) correlates with the outcome. The aim of this study is to analyze factors related to mortality and morbidity, according to the technique of abdominal closure of these neonates. Retrospective analysis of charts from 76 consecutive neonates with AWD treated in a single institution. They were divided according to the type of abdominal wall closure: group I: primary closure, group II: silo followed by primary closure and group III: silo followed by polypropylene mesh. Outcome was analyzed separately for neonates with gastroschisis and omphalocele. There were 13 deaths (17.1%). Mortality for neonates with isolated defects was 9.6%. Mortality rate was similar in all groups for either neonates with gastroschisis or omphalocele. Postoperative complications were not significantly different among groups except for a prolonged time of hospitalization in group III. Mortality rate is not correlated with the type of abdominal closure. Neonates with primary closure or with other methods of abdominal wall closure had similar rate of postoperative complications. Neonates with mesh closure of the abdomen have prolonged hospitalization. The use of a polypropylene mesh is a good alternative for neonates whose primary closure or closure after silo placement is not possible.

Prenatal diagnosis and perinatal outcome of 38 cases with congenital diaphragmatic hernia: 8-year experience of a tertiary Brazilian center

OBJETIVO: Avaliar os resultados neonatais dos casos de hérnia diafragmática congênita com diagnóstico pré-natal.PACIENTES E MÉTODOS: De janeiro de 1995 a dezembro de 2003 foram revisados os dados de 38 fetos com hérnia diafragmática diagnosticada durante o período pré-natal na Unidade de Medicina Fetal do Departamento de Obstetrícia e Ginecologia da Faculdade de Medicina da Universidade de São Paulo. Os dados pré-natais analisados foram: idade gestacional no diagnóstico, cariótipo fetal, lado da lesão, presença de malformação estrutural associada, herniação hepática e desvio garve de mediastino. Os dados perinatais foram avaliados consultando os prontuários médicos ou por contato telefônico. RESULTADOS: A idade gestacional média no diagnóstico foi de 29 semanas (16-37). Trinta (79%) casos apresentavam lesão à esquerda e 8 (21%) à direita. Malformações estruturais associadas foram observadas em 21 (55%) casos, dos quais 12 fetos apresentaram cariótipo normal, enquanto 9 exibiram anomalias cromossômicas. Hérnia diafragmática isolada foi identificada em 17 (457%) casos. A taxa de mortalidade geral foi de 92%. A taxa de óbito fetal, neomorto precoce, neomorto tardio e sobrevivente após 28 dias de vida foram respectivamente: (i) para o grupo com malformação estrutural associada e cariótipo normal, de 42%, 50%, 0% e 8%; (ii) para os casos com cromossomopatia, de 56%, 44%, 0% e 0%; (iii) para os casos com lesões isoladas de 0%, 76%, 12% e 12%. Na hérnia diafragmática congênita isolada, a mortalidade neonatal foi de 88%. CONCLUSÃO: Em nosso serviço, a mortalidade perinatal nos casos de hérnia diafragmática diagnosticada durante o pré-natal é muito alta. Óbitos neonatais precoces estão associados com presença de outros defeitos estruturais ou anomalias cromossômicas. Nos casos de HDC isolada, a mortalidade está relacionada à presença de fígado herniado, lado direito da lesão e desvio grave de mediastino.

Onfalocele: prognóstico fetal em 51 casos com diagnóstico pré-natal / Omphalocele: fetal prognosis in 51 cases with prenatal diagnosis

Objetivo: avaliar o prognóstico fetal dos casos de onfalocele com diagnóstico pré-natal. Métodos: foram analisados 51 casos de onfalocele com diagnóstico pré-natal e divididos em 3 grupos: Grupo 1, onfalocele isolada; Grupo 2, onfalocele com malformações estruturais associadas e cariótipo normal; Grupo 3, onfalocele associada à cromossomopatia. As análises foram realizadas em relação à sobrevida geral e pós-correção cirúrgica, considerando as malformações associadas, idade gestacional no parto, peso no nascimento e tamanho da onfalocele. Resultados: o Grupo 1 correspondeu a 21 por cento (n=11), o Grupo 2 a 55 por cento (n=28) e o Grupo 3 a 24 por cento (n=12). Todos os casos do grupo 3 evoluíram para óbito, e a cromossomopatia mais freqüente foi a trissomia do 18. A sobrevida foi de 80 por cento no Grupo 1 e de 25 por cento no Grupo 2. Dezesseis casos foram submetidos à correção cirúrgica (10 isoladas e 6 associadas) e 81 por cento sobreviveram (8 isoladas e 5 associadas). A mediana do peso no nascimento dos sobreviventes pós-correção cirúrgica foi 3.140 g e dos que morreram foi de 2.000 g (p=0,148) e a idade gestacional do parto foi de 37 e de 36 semanas (p=0,836), respectivamente. A relação das circunferências onfalocele/abdominal diminuiu com a idade gestacional, 0,88 entre 25-29 semanas e 0,65 entre 30-35 semanas (p=0,043). Não foi observada diferença significativa no tamanho da onfalocele nos 3 grupos (p=0,988) e influência deste prognóstico pós-correção cirúrgica (p=0,553). Conclusão: a sobrevida geral e pós-correção cirúrgica foi de 25 e 81 por cento, respectivamente. As malformações associadas representam o principal fator prognóstico das onfaloceles com diagnóstico pré-natal, visto que se associam com prematuridade e baixo peso.

Gastrosquise: avaliação pré-natal dos fatores prognósticos para sobrevida pós-natal / Gastroschisis: prenatal evaluation of prognostic factors for postnatal outcome

Objetivo: avaliar a evolução de 24 casos de gastrosquise, em relação aos fatores prognósticos pré-natais que interferiram na sobrevida pós-natal. Pacientes e Métodos: foram avaliados 24 casos de gastrosquise diagnosticados no Setor de Medicina Fetal do Hospital das Clínicas da FMUSP, durante um período de 8 anos. Foram classificados em gastrosquise associada, quando presentes outras malformações, e isolada. Nos dois grupos foram analisados parâmetros referentes às alças intestinais dilatadas na avaliação ultra-sonográfica (dilatação > ou igual 18 mm), complicações obstétricas e resultados pós-natal. Resultados: foram observados 9 casos de gastrosquise associada (37,5 por cento) e 15 casos de gastrosquise isolada (62,5 por cento). Todos os casos de gastrosquise associada foram de prognóstico letal, levando a uma alta taxa de mortalidade geral de 60,8 por cento. Do grupo de gastrosquises isoladas, todos nasceram vivos e foram submetidos a cirurgia, com taxa de sobrevida de 60 por cento e mortalidade pós-natal de 40 por cento. A mediana da idade gestacional foi de 35 semanas e o peso no nascimento de 2.365 gramas no grupo geral. Nas gastrosquises isoladas, o parto prematuro ocorreu em 10 casos, principalmente decorrente de complicações obstétricas. Dois recém-nascidos foram considerados pequenos para a idade gestacional e apenas 3 apresentaram peso no nascimento > ou igual 2.500 gramas. O oligoidrâmnio foi um achado comum (46,6 por cento), sendo mais freqüente no grupo que evoluiu para óbito neonatal (66,7 por cento). A avaliação ultra-sonográflca das alças intestinais demonstrou que em 13 de 15 casos (86,6 por cento) as alças eram dilatadas, mas sem relação significativa com o prognóstico e achados pós-natais. Não houve diferença significativa em relação a idade gestacional e peso no nascimento, comparando os grupos de vivos e óbitos neonatais. Conclusões: as gastrosquises isoladas apresentam um melhor prognóstico quando comparadas às associadas, sendo de suma importância a sua diferenciação pré-natal. As gastrosquises isoladas estão associadas a complicações obstétricas (60 por cento), prematuridade e baixo peso ao nascimento. O diagnóstico pré-natal permite uma melhor monitorização das condições fetais. O parto destas gestações deve ser no termo, a menos que complicações obstétricas se apresentem.