a 21yr primi , 28weeks , USG shows , holoprosencephaly, cleft lip with cleft palate. further management?



[1:57pm, 1/30/2016] Good night: Semilobar holoprosencephaly is characterized by an incomplete forebrain division, resulting in partial separation of the cerebral hemispheres, typically posteriorly. In lobar holoprosencephaly (seen in the image below), there is complete ventricular separation, with focal areas of incomplete cortical division or anterior falcine hypoplasia present. [2:01pm, 1/30/2016] Good night: At prenatal ultrasonography, primary CNS findings include the following: (1) single, sickle-shaped or horseshoe monoventricle; (2) absent midline echo due to the absence of an interhemispheric fissure, falx, corpus callosum, and septum pellucidum; (3) thin cortical rim; (4) single, fused thalamus; (5) microcephaly; AQ plan plan pl ppp ventriculomegaly/hydrocephalus. Associated craniofacial ultrasonographic findings include the following: (1) ocular hypotelorism or cyclopia, (2)4 proboscis or abnormal nasal bone formation, and (3) cleft lip and/or palate (midline or bilateral). Postnatally, alobar holoprosencephaly shows a single% ventricle, fused thalami, and a thin, usually poorly differentiated cortical mantle. A dorsal cyst or ventricular remnant may also be detected. Semilobar holoprosencephaly is detected by identifying the partial cleavage of the occipital horns and the presence of a posterior falx and a posterior portion of the corpus callosum. Anteriorly, there is typically fusion of the ventricles and of the hemispheres, with concomitant absence of the corpus callosum and of the septum pellucidum. Lobar holoprosencephaly is a continuum. In some patients, the ultrasonographic images show fusion of the anterior horns and of portions of the frontal lobes. The mildest forms of lobar holoprosencephaly may be manifested only by absence of the septum pellucidum. Sonograms of the middle interhemispheric fusion variant demonstrate normal cleavage anteriorly and posteriorly, with fusion of the hemispheres and absence of the body of the corpus callosum in the posterior, frontal, and parietal regions.

For each patient, pediatricians should follow a diagnostic protocol including dysmorphology examination, complete family history and ascertainment of risk factors, and neuroimaging. Many medical issues, including hypothalamic dysfunction, endocrinologic dysfunction, motor impairment, respiratory issues, seizures, and hydrocephalus should be prioritized in management. Pediatricians should work with genetic specialists to identify syndromic forms and to perform cytogenetic investigation, molecular screening, and genetic counseling in order to fully characterize prognosis and recurrence risk.

if such pt.comes to u with bleeding/leaking/early labour pain? management termination.yes legally it is against law but with humanity ground take pt.in faith and indicate indication and can terminate concise ring labour pt if u want.

if we want to terminate pregnancy gynecologist have many reason to terminate pregnancy.better to terminate pregnancy to prevent psychological trauma to patient at term.

agree with friends suggesting termination. get a proper informed consent. better to terminate than prolong the agony of the mother. labour at this time is less traumatic. and who knows.. at term we also run a higher risk of envountering indications for lscs etc. sobi strongly suggest termination

After birth treat as per rule of ten.

There is no point in continuing the pregnancy. Induce labour and terminate the pregnancy. Knowingly you need not continue the pregnancy. Explain to the patient, guardian attendants. Take informed consent.

In my personal view explain the prognosis to pt n attendant and then let them decide what they want. Many a times they R not able for such expensive treatment afterwards. so they should decide by their own whether to continue or terminate d pregnancy

so unfortunate....but hv to continue pregnancy. .

Continue pregnancy what else I don't think so it's legal for 28 weeks pregnancy for abortion .

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