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A prenatal study of fetal platelet count and size with application to fetus at risk for Wiskott-Aldrich syndrome.

Platelet counts and platelet size were determined in midtrimester fetuses to obtain reference values to be used in prenatal diagnosis of inheritable platelet disorders. In 17- to 21-week fetuses, platelet counts ranged between 135 and 283 x 10(9)/L, and thus did not differ from those of newborn infants and adults. Mean platelet volume fell between 6.0 and 7.0 fl, which is in the range for adults. The data were used to exclude Wiskott-Aldrich syndrome in an 18-week fetus at 50% risk of being affected.

Blood Coagulation Disorders↗

Natural protective mechanisms against endoscopic white-light injury in the fetal lamb eye.

OBJECTIVE: To evaluate light transmission, possible light trauma, and techniques for protection of the fetal eyes during intrauterine videoendoscopic surgery in a sheep model. METHODS: In vitro studies were done at various gestational ages, including spectrometry of light output by a halogen light source and telescope and light transmission by spectrophotometry in the range of 180-3000 nm through amniotic fluid (AF) and fetal eyelids. In vivo electron-microscopic, morphologic analysis of the retinas of 65-, 95-, 108-, and 112-day-old fetal lambs with (n = 8) and without (n = 8) 30 minutes' light exposure to the open eye was also done. RESULTS: The light spectrum at the tip of the telescope was 400-750 nm, with a maximum irradiance of 3x10(-3) W/cm2 at 580 nm. In the ultraviolet spectrum (less than 300 nm), irradiance was less than 0.5x10(-3) W/cm2. Light transmission through ovine AF ranged from 30% at 300 nm to 89% at 700 nm at 84 days' gestation, decreasing to less than 0.01% (300 nm) and 70% (700 nm) at 112 days. Fetal eyelids did not transmit more than 1% of light (any wavelength). After direct in vivo light exposure, no retinal damage was found. Photoreceptors were present from 108 days onward, but chromophores were scant or absent at all ages studied. CONCLUSION: The light spectrum of a standard endoscope is limited to 400-750 nm; ultraviolet light is filtered out. The AF transmits harmful blue light poorly. Fetal eyelids seem to protect the eye by extremely low transmission and light dispersion. Even with the eye open, no morphologic retinal damage was found. The strong light sources used with fetal endoscopy did not appear to pose a threat to the fetal retina.

Animals↗

Abdominal wall hernia: an uncommon complication of in utero vesicoamniotic shunt placement.

Three cases are reported in which placement of a vesicoamniotic shunt in utero for the treatment of obstructive uropathy led to the rare complication of abdominal wall hernia. All 3 patients underwent vesicoamniotic shunt placement for severe oligohydramnios and a markedly dilated bladder in an effort to preserve renal function and to prevent pulmonary hypoplasia. All three shunts were initially placed at or above the umbilicus. The abdominal wall hernias were closed postnatally at the time of temporary vesicostomy for urethral obstruction. All 3 patients had sufficient pulmonary development, but 2 of 3 had renal failure, requiring dialysis. Management and potential etiology of this rare complication are discussed.

Amnion↗

Antenatal intervention: jeopardizing life or limb?

The association of limb loss with antenatal urologic intervention has recently been questioned. At present, no such cases have been reported. We present a case of antenatal vesicoamniotic shunting for posterior urethral valves in which the shunt entered the right lower extremity and coursed subcutaneously over the femoral triangle and inguinal canal and into the bladder. Although the limb was intact, we present images and a discussion of the potential for extremity injury with antenatal urologic intervention.

Adult↗

Congenital diaphragmatic hernia: advances in prenatal therapy.

Despite intensive clinical and experimental efforts, mortality from CDH remains high. More than two decades of research in multiple centers has led to a better understanding of the pathophysiology, prognosis, and treatment options for fetuses that have CDH. It now appears that fetuses that have prenatally diagnosed CDH can be stratified into high- and low-risk groups based upon sonographic parameters. Fetuses that do not have liver herniation into the chest that have a favorable LHR have an excellent chance of survival with postnatal therapy. Prenatal diagnosis allows the time and place of delivery to be planned in advance so these infants can be treated in a tertiary care nursery that has maximal medical and surgical therapy. Fetuses that have liver herniation into the chest and an unfavorable LHR have a grim prognosis. These fetuses might benefit from in utero intervention. There is no role for open fetal repair of the diaphragmatic detect; however, fetoscopic temporary tracheal occlusion might improve lung growth and development and might decrease morbidity and mortality in these infants. The FETENDO strategy appears to work, and for the first time it offers hope to the fetus that has high-risk CDH, but its efficacy must be proven in a proper randomized, controlled trial.

Animals↗

In-utero treatment of fetal bladder-outlet obstruction by a ureterocele.

Fetal bladder-outlet obstruction by ureteroceles is usually treated at birth. However, such obstruction may be detrimental to the health of the fetus and so in-utero correction is preferable. We describe the successful cytoscopy guided laser incision, with no complications, of a uterocele that was causing bladder-outlet obstruction in a fetus of 19 weeks and 6 days gestation.

Adult↗