Cases reported "Fetal Diseases"

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1/184. The outcome after perinatal management of infants with potential airway obstruction.

    Masses in the head and neck are being detected prenatally with increasing frequency, necessitating the need for management of potential upper airway obstruction at delivery. Establishment of the airway at delivery and its maintenance thereafter are critical. This should optimally be performed with the baby still attached to the placental circulation. The importance of multidisciplinary team management, including a high risk obstetrician, neonatologist, pediatric otolaryngologist, pediatric thoracic surgeon, and an anesthetist, cannot be overemphasized. Endotracheal intubation is attempted first, if unsuccessful then is followed by insertion of a rigid bronchoscope. tracheotomy should be reserved for airway obstructions, which are not amenable to endotracheal intubation or in babies in whom exchange from a bronchoscope to endotracheal tube cannot be safely performed. The management of six infants with prenatally diagnosed potential airway obstruction is presented. morbidity and mortality still ultimately depend on the severity of the existent anomalies.
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ranking = 1
keywords = airway obstruction, obstruction, airway
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2/184. hydronephrosis in pregnancy: simultaneous depiction of fetal and maternal hydronephrosis by magnetic resonance urography.

    Magnetic resonance urographic (MRU) techniques possess image quality and diagnostic capability that are improving with increasingly sophisticated imaging sequences and shorter scanning times. We describe the application of a fast breath-hold MR sequence (HASTE) in the assessment of ureteric obstruction in pregnancy. In the patient presented, HASTE MRU was successful in depicting ureteral anatomy and demonstrated dilation of both ureters below the level of the pelvic brim. This observation suggested distal ureteral obstruction rather than simple hydronephrosis of pregnancy. As a result, bilateral nephrostomies were performed and neonatal prematurity was avoided. Interestingly, in this patient, HASTE MR imaging also showed evidence of concurrent fetal hydronephrosis.
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ranking = 0.060163735656746
keywords = obstruction
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3/184. Definitive diagnosis of intestinal volvulus in utero.

    Midgut volvulus with or without intestinal malrotation can occur in fetal life. Several reports have described congenital midgut volvulus showing non-specific sonographic findings of intestinal obstruction and perforation in utero. None of the previously reported cases, however, were definitively diagnosed as midgut volvulus by fetal sonography. We report two cases both exhibiting the sonographic 'whirlpool' sign, in utero. color Doppler interrogation provided a clue to the viability of the involved intestinal segment.
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ranking = 0.030081867828373
keywords = obstruction
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4/184. Antenatal sonographic diagnosis of epignathus at 15 weeks of pregnancy.

    Epignathus is a rare, benign, congenital teratoma of the hard palate. Most of these teratomas are unidirectional and protrude through the mouth. Hence, the prognosis depends on the size of the tumor and the degree of face distortion and airway obstruction that it causes. However, some epignathi protrude bidirectionally, involving and destroying the brain tissue, resulting in a poor prognosis. This report presents a case of ultrasonographic detection of a bidirectional epignathus at 15 weeks of pregnancy.
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ranking = 0.14173413469028
keywords = airway obstruction, obstruction, airway
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5/184. Successful management of fetal cervical teratoma using the EXIT procedure.

    Fetal cervical teratoma is a cause of polyhydramnios, premature labor, and newborn airway obstruction. Formation of a multispecialty team and use of the EXIT procedure is essential for survival of the neonate. Without a team, there is little hope for fetal survival; mortality will be 80-100%. early diagnosis and planning are essential. Cervical teratomas can contribute to pulmonary insufficiency and chondromalacia because of a mass effect in utero and underdevelopment of the fetal lungs.
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ranking = 0.14173413469028
keywords = airway obstruction, obstruction, airway
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6/184. prenatal diagnosis of cystic bladder distension secondary to obstructive uropathy.

    We report the perinatal findings of a huge midline posterior cystic bladder distension secondary to lower urinary tract obstruction and prune-belly syndrome in a male fetus. A 40-year-old woman, gravida 3, para 0, was referred at 21 gestation weeks with sonographic findings of anhydramnios and a fetus with a 9.5 x 6.0 cm intra-abdominal cystic mass containing two chambers. The in utero ultrasound-guided fetal bladder drainage using a single needle aspiration and the ultrasound follow-ups of fetal bladder filling provided a diagnostic aid. This method helped to show the position of the bladder and the cystic bladder mass as well as the status of communication in response to decompression or filling of the fetal bladder. cytogenetic analysis revealed a 46,XY karyotype. autopsy showed agenesis of the posterior urethra, prominent megacystis, a cystically distended mass arising from the lower posterior bladder, hydronephrosis, megaureters, and anorectal agenesis with an intestinal blind end adherent to the posterior wall of the uterus. There were no urogenital duplication, hindgut duplication, or urachal abnormalities. The contracted bladder had a full-thickness muscular wall with a trigone and two ureteral orifices while the cystically distended bladder did not have any opening and was lined by a very thin wall. histology of the cystic bladder wall demonstrated typical urothelium, lamina propria and muscularis propria. The pathogenesis and differential diagnosis of cystic bladder distension are discussed.
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ranking = 0.030081867828373
keywords = obstruction
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7/184. prenatal diagnosis of tracheal obstruction: possible association with maternal pertussis infection.

    A fetus with the sonographic appearance of echogenic and enlarged lungs and dilated trachea and bronchi, indicating laryngotracheal obstruction, is reported. Additionally, the fetus had ascites and subcutaneous edema and the amniotic fluid volume was reduced. Doppler flow investigation of the systemic venous circulation revealed signs of heart failure, and color Doppler visualized possible increased pulmonary flow. Following termination of pregnancy, autopsy confirmed the sonographic observations and revealed a hypoplastic thymus. During the present pregnancy the mother suffered from sustained cough, and serological tests revealed acute pertussis infection. polymerase chain reaction investigation for bordetella pertussis in the amniotic fluid was negative. The possibilities of pertussis toxins as noxious factors and of an atypical presentation of DiGeorge anomaly are discussed.
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ranking = 0.15040933914187
keywords = obstruction
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8/184. In utero repair of rectal atresia after complete resection of a sacrococcygeal teratoma.

    PURPOSE: A case of a fetus with a prenatally diagnosed sacrococcygeal teratoma that produced high-output cardiac failure, hydrops, rectal atresia, and urinary tract obstruction is presented. The unique prenatal surgical management along with the embryogenesis of tumor-related rectal atresia is discussed. CASE REPORT: A large fetal sacrococcygeal teratoma with a significant intrapelvic component was detected at routine ultrasound in a 35-year-old gravida 3 para 2. Fetal hydrops developed rapidly due to high-output cardiac failure from the vascular 'steal' by the growing tumor. The urinary tract was obstructed due to the intrapelvic tumors mass. At 27 weeks' gestation, the female fetus underwent hysterotomy, resection of the entire mass and urinary diversion via bilateral flank ureterostomies. The rectum was found to be completely atretic due to apparent encasement by the tumor. Pull-through anorectoplasty was carried out concurrently. At 30 weeks' gestation, the mother developed preterm labor and a 1.8-kg was delivered by cesarean section. The baby did very well for 3 days but had a cardiac arrest and died due to an atrial perforation by a transfemoral venous catheter. CONCLUSIONS: To our knowledge this is the first report of a complete prenatal resection of a sacrococcygeal teratoma with concomitant pull-through anorectoplasty for rectal atresia.
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ranking = 0.030081867828373
keywords = obstruction
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9/184. Functional urinary tract obstruction developing in fetuses with isolated gastroschisis.

    OBJECTIVE: To evaluate the frequency and natural history of urinary tract abnormalities developing in fetuses presenting with initially isolated gastroschisis. methods: Serial ultrasounds were performed prospectively on fetuses identified by our prenatal diagnosis program as having a gastroschisis. When abnormalities in the urinary tract were identified prenatally, newborns were evaluated by a pediatric urologist. RESULTS: Over a 1-year period four out of 12 fetuses with gastroschisis developed deformations of the urinary tract. In three fetuses the bladder herniated through the abdominal wall defect. Two also had upper tract dilatation. A fourth fetus developed bilateral hydronephrosis with a normally situated bladder. Once the gastroschisis was repaired none of the newborns had evidence of structural obstruction of the urinary tract, however, hydronephrosis with or without reflux persisted for several months. CONCLUSIONS: Deformations of the fetal urinary tract can develop secondary to gastroschisis. They do not appear to represent separate malformations and evaluation with fetal karyotyping may not be indicated. When hydronephrosis is present ongoing urologic evaluation of the neonate is indicated.
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ranking = 0.15040933914187
keywords = obstruction
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10/184. prenatal diagnosis of airway compromise: EXIT (ex utero intra-partum treatment) and foetal airway surgery.

    Four cases of potential airway obstruction diagnosed in the early antenatal period are presented. Their management is outlined, and the need for a multidisciplinary team approach to these problems is highlighted. The experience of the ex-utero intrapartum treatment (EXIT) procedure is presented, and the first attempt at intra-uterine tracheal surgery is introduced.
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ranking = 0.2046225920348
keywords = airway obstruction, obstruction, airway
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