Cases reported "Fetal Diseases"

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1/66. Fetal neurenteric cyst causing hydrops: case report and review of the literature.

    Routine prenatal ultrasound revealed a unilocular cystic mass associated with upper thoracic hemivertebrae that grew to 6 cm at 28 weeks and was associated with hydrops. A thoraco-amniotic shunt decompressed the cyst and resolved the hydrops, but the shunt occluded 17 days later. Preterm labour led to vaginal delivery at 31 2/7 weeks. Postnatally, the cyst was decompressed by thoracentesis due to respiratory distress. It was resected on day four of life. Severe tracheobronchomalacia was present post-operatively, presumably due to prenatal mass effect of the cyst. At one year of age, the child has recovered completely without adverse respiratory or neurological sequelae.
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ranking = 1
keywords = operative
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2/66. Congenital nasal hemangiopericytoma: intrauterine, intraoperative, and histologic findings.

    hemangiopericytoma is a rare tumor of mesenchymal origin. To date, 91 cases of nasal or paranasal hemangiopericytoma and 59 congenital hemangiopericytomas have been reported in the literature. A congenital hemangiopericytoma arising from the nasal cavity and skull base has not yet been described. We report a case of a male newborn with a highly vascular nasal tumor diagnosed by in utero sonography with three-dimensional surface reconstruction. The tumor extended to the right anterior skull base, the right nasal cavity, and the right side of the nasal pyramid. A complete resection by neodymium:yttrium-aluminum-garnet-potassium titanyl phosphate ("Nd:YAG-KTP") laser was performed on the day of cesarean section at 33 weeks' gestation. The tumor was diagnosed as hemangiopericytoma by histologic and immunohistochemical findings. Postoperative nasal flow, feeding, and sight were unimpaired. At the 9-month follow-up, the infant remained free of disease.
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ranking = 5
keywords = operative
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3/66. Laparoscopic diagnosis and management of ovarian torsion in the newborn.

    BACKGROUND AND OBJECTIVES: The application of laparoscopic techniques in the surgical management of neonatal ovarian cysts is proving valuable both as a diagnostic tool and a potential therapeutic intervention. We report the successful management of a prenatally diagnosed ovarian cyst in a newborn female and provide operative evidence for the presumptive etiology of the cyst. methods AND RESULTS: A prenatally diagnosed ovarian cyst was managed using 5 mm laparoscopic instruments in a newborn female. The prenatal ultrasonographic and operative findings are consistent with in utero adnexal torsion with subsequent autoamputation and cystic degeneration of the ovary. The orphaned ovarian cyst was removed from the infant's abdominal cavity by enlarging the camera port incision. DISCUSSION: The application of laparoendoscopic procedures in infants and children continues to evolve with the availability, of microinstrumentation and increasing experience among pediatric surgeons. This approach may prove valuable in the diagnosis and management of prenatally diagnosed ovarian cysts. In addition, further insight into the etiology of congenital ovarian cysts may be obtained. The safety and efficacy of this approach in these infants remains to be fully evaluated.
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ranking = 2
keywords = operative
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4/66. esophageal atresia: primary repair of a rare long gap variant involving distal pouch mobilization.

    This report describes a rare type IIIb variant of long gap pure esophageal atresia treated with delayed primary repair. Preoperative imaging showed a distal pouch with a unusually long tapering end, and a 5-vertebral body gap. Delayed exploration with mobilization of the distal pouch allowed for successful primary repair without the need of myotomy. The characteristic radiological appearance of this rare variant enables its preoperative recognition and warrants attempting primary repair with a good expectation of success.
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ranking = 2
keywords = operative
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5/66. Ultrasonographic detection of intrauterine intussusception resulting in ileal atresia complicated by meconium peritonitis.

    A neonate with ileal atresia (IA) complicated by meconium peritonitis (MP) whose prenatal ultrasonography (US) detected an intrauterine intussusception (IUI) is reported. Fetal ascites, dilated bowel loops, and abdominal calcifications were identified on serial US from 25 weeks of gestation. Intestinal loops with high echogenecity and a "target-like" appearance suggestive of IUI were detected in the right lower quadrant. The 2,680-g male was delivered vaginally at term and underwent a laparotomy. Fibrous adhesions and small calcifications were scattered throughout the peritoneal cavity. IA (interrupted type) was confirmed 17.0 cm cranial to the ileocecal valve (ICV). An ileo-ileal intussusception was also found between 16.5 cm and 9.0 cm cranial to the ICV. Partial resection of the ileum and an ileo-ileal anastomosis was performed. The postoperative course was uneventful. In this case, the pathological process of IUI resulting in IA and MP was demonstrated sonographically by identifying the "target-like" appearance in the fetus.
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ranking = 1
keywords = operative
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6/66. Transabdominal amnioinfusion to avoid fetal demise and intestinal damage in fetuses with gastroschisis and severe oligohydramnios.

    BACKGROUND/PURPOSE: Despite dramatic improvement in survival rate for neonates with gastroschisis, significant postoperative morbidity and a low mortality rate still occur. Furthermore, even in recent publications, some fetal death has been reported. Does this mean that antenatal diagnosis of gastroschisis is a missed opportunity? In fact, decreased amniotic fluid (AF) volume is observed in some fetuses with gastroschisis. However, oligohydramnios is associated with an increased risk of fetal suffering. When severe oligohydramnios is observed, intrapartum amnioinfusion, to restore AF volume, may help avoid fetal complications. methods: Two fetuses with gastroschisis and severe oligohydramnios were treated antenatally with amnioinfusion of saline solution. In one case, fetal heart beat decelerations were observed at 27 weeks' gestation among with the oligohydroamnios and serial transabdominal amnioinfusions were performed. In the second case, severe oligohydramnios was observed at 31, weeks and an amnioinfusion was performed. The 2 babies were delivered at 31 and 34 weeks, respectively. RESULTS: In both cases, exteriorized bowel was nearly normal at birth, and primary closure could be performed. Outcome was favorable, and they were discharged home on day 43 and day 54, respectively. CONCLUSIONS: Because fetuses with gastroschisis and oligohydramnios are part of a particular high-risk group, serial ultrasound examination and computerized fetal heart beat monitoring are necessary during the third trimester. In selected cases of gastroschisis associated with severe oligohydramnios, serial amnioinfusion may be required.
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ranking = 1
keywords = operative
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7/66. Prenatal sonographic diagnosis of uterine rupture following open fetal surgery.

    BACKGROUND: Reported cases of uterine rupture diagnosed by ultrasound have shown fetal membranes ballooning through uterine rupture sites, or adjacent areas of hemorrhage. CASE: A 27-year-old gravida 3, para 2 had open fetal surgery to repair a fetal myelomeningocele at 28 weeks' gestation. Her postoperative course was complicated by threatened preterm labor and anhydramnios. At 33 weeks' gestation, with maternal symptoms of bowel obstruction, ultrasound showed a fetal leg and section of umbilical cord protruding through the uterine wall. CONCLUSION: Even in the presence of anhydramnios, uterine wall rupture was identified, because ultrasound evaluation of the uterine wall showed prolapsed fetal parts and umbilical cord. Persistent anhydramnios after open fetal surgery should prompt a search for uterine rupture.
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ranking = 1
keywords = operative
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8/66. Laparoscopic management of neonatal ovarian cysts.

    A laparoscopic approach was used in four infants with antenatally diagnosed ovarian cysts requiring surgical intervention. Operating time ranged from 60 to 90 minutes, there were no intraoperative or postoperative complications, and all infants were discharged within 23 hours after surgery. If performed by experienced practitioners, microendoscopy is an alternative to laparotomy in neonates requiring surgical intervention for ovarian cysts.
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ranking = 2
keywords = operative
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9/66. Successful fetal sacrococcygeal teratoma resection in a hydropic fetus.

    BACKGROUND/PURPOSE: The development of hydrops in a fetus with a sacroccocygeal teratoma (SCT) usually is a predictor of fetal demise; in utero resection may offer the only chance of survival. Although the authors had performed this procedure in 3 previous cases, they had no long-term patient survival. The authors report a successful case of in utero resection of a fetal sacrococcygeal teratoma. methods: The authors resected a fetal SCT from a 23-weeks-gestation hydropic fetus, using gradually tightening umbilical tapes at the tumor base, electrocautery, and careful sharp dissection. After a blood transfusion, the fetus suffered cardiac arrest but was resuscitated and returned to the uterus. RESULTS: Postoperatively, residual SCT growth ceased, and hydrops rapidly resolved. Five weeks after the procedure, the infant was delivered because of preterm labor, and, after resection of residual SCT, was discharged home at 3 months of age. She is now a healthy 3 year old. CONCLUSION: This case shows that successful fetal SCT resection and long-term patient survival is possible.
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ranking = 1
keywords = operative
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10/66. Antenatal diagnosis of congenital hepatoblastoma in utero.

    A fetus with a huge hepatic tumor was detected by sonography at 36 weeks of gestation. The mass appeared as a single, solid and polylobular tumor located in the right lobe of the liver. Foci of hemorrhage, necrosis and some tiny calcifications were seen. The adjacent right kidney appeared normal but was displaced. The right adrenal gland was not visualized. Three-dimensional power Doppler sonography further depicted the corresponding vascular anatomy of the tumor, including its vascularization pattern and blood supply. The tumor was situated to the right of the umbilical vein and portal sinus, possibly deriving its blood supply from the portal circulation. The fundamental findings suggested the diagnosis of hepatoblastoma by exclusion of other possibilities. The baby was delivered by cesarean section at 36 weeks' gestation, due to signs of fetal distress. Unfortunately, hypotension, tachycardia, and tachypnea developed shortly after birth. Surgical intervention was performed, but intractable bleeding occurred intra-operatively. The infant died at 6 days of age. autopsy confirmed the diagnosis of hepatoblastoma. We believe this is the first reported case of the antenatal diagnosis of congenital hepatoblastoma.
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ranking = 1
keywords = operative
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