Dear Colleagues

I wish you a very happy Holi

In this issue one of my neurosurgeon friend posted a topic on Neural tube Defect. In fact this topic was discussed by him in our ObGy journal Club. It was a different perspective of neural tube defect and spina bifida we got from him. I felt that I should share this knowledge with you and requested him to get a small writing on this . We are very happy that a specialist from different faculty contributed in this newsletter .

Second topic is a personal experience of ovarian hyperstimulation of severe variety. It was a nightmare for us. The same dose we used to stimulate the ovaries was used ,but this time, it made the ovaries so angry that it took 12 days of intensive care ,that saved her life.

Our Journal club is well appreciated by the doctors practicing in city . We discussed gestational trophoblastic disease and its genetics in detail. The meeting was chaired by Dr.Shashi Khare, head of the deptt Ob Gy, Jabalpur Medical College.Our next topic is a case discussion of “Cesarian Hysterectomy in a case of Placenta Previa Type IV,with consumptive coagulopathy” .If you wish to attend please inform, every body is welcome without any registration fees.

With best wishes

Dr.D’Pankar Banerji

1. Cenal Nervous System Abnormalities during Fetal Life & their Management Strategy

Dr Asheesh Tandon      MCh Neurosurgery (AIIMS)

Brain Tumor Fellow TWH Toronto Canada

Fellow Minimally Invasive Neurosurgery POW Sydney Australia

Neural Tube defects.

These include anencephaly, spina bifida and cephalocele.

Etiology – Precise etiology for majority is unknown. However chromosomal abnormalities, single mutant genes, maternal diabetes mellitus or teratogens such as antiepileptic drugs are implicated in10% cases. If parent or previous sibling has had neural tube defect the risk of recurrence is 5-10%. Periconceptual supplementation of the maternal diet with folate reduces by about half, the risk of developing these defects

Anencephaly – Absence of the cranial vault (acrania) with secondary degeneration of the brain. Diagnosis can be made after 11 week. Anencephaly is fatal at or within hours of birth.

Spina Bifida (SB)

The neural arch, usually in the lumbosacral region, is incomplete with secondary damage to the exposed nerves. Diagnosis of SB requires the examination of each neural arch from the cervical to the sacral region both transversely and longitudinally. In the transverse scan the arch is "U" shaped and there is an associated bulging meningocele (thin-walled cyst) or myelomeningocoele. The diagnosis of SB is enhanced if associated abnormalities like Arnold Chiari malformations ACM are detected. Abnormalities in ACM  include frontal bone scalloping (lemon sign), and obliteration of the cisterna magna with either an "absent" cerebellum or abnormal anterior curvature of the cerebellar hemispheres (banana sign). Variable degree of hydrocephalus is also appreciated. Alpha Fetoprotein levels at 16 wks of pregnancy may also help pick up such cases

. In spina bifida the surviving infants suffer from variable degree of neurological compromise predominantly weakness in the lower limbs and incontinence; however intelligence may be normal.There is some experimental evidence that in-utero closure of spina bifida may reduce the risk of handicap because the amniotic fluid in the third trimester is thought to be neurotoxic.

Infants and children receiving early treatment generally have good outcome especially if they do not suffer from severe neurological deficits

Encephalocele

Encephaloceles are recognized as cranial defects with herniated fluid‑filled or brain‑filled cysts. They are most commonly found in an occipital location (75% of the cases) but alternative sites include the frontoethmoidal and parietal regions.In cephalocele the prognosis is inversely related to the amount of herniated cerebral tissue. Early surgery in neurologically preserved babies leads to relatively good prognosis.

Hydrocephalus

In hydrocephalus there is pathological increase in the size of the cerebral ventricles. This may result from chromosomal and genetic abnormalities, intrauterine hemorrhage or congenital infection, although many cases have as yet no clear‑cut etiology. A transverse scan of the fetal head at the level of the cavum septum pellucidum will demonstrate the dilated lateral ventricles, defined by a diameter of 10 mm or more.

Prognosis - Fetal or perinatal death and neurodevelopment in survivors are strongly related to the presence of other malformations and chromosomal defects. Even in mild ventriculomegaly about 10% of cases mild to moderate neurodevelopmental delay has been reported.

Agenesis of Corpus Callosum

Agenesis of the corpus callosum may be either complete or partial (usually affecting the posterior part). It is commonly associated with chromosomal abnormalities (usually trisomies 18, 13 and 8). This depends on the underlying cause. In about 90% of those with apparently isolated agenesis of the corpus callosum development is normal.

Dandy Walker Malformation

The Dandy-Walker complex refers to a spectrum of abnormalities of the cerebellar vermis, cystic dilation of the fourth ventricle and enlargement of the cisterna magna. Chromosomal abnormalities (usually trisomy 18 or 13 and triploidy), congenital infection or teratogens such as warfarin are reported etiological factors, but it can also be an isolated finding. Dandy-Walker malformation is associated with a high postnatal mortality (about 20%) and a high incidence (more than 50%) of impaired intellectual and neurological development.

Arachnoid Cyst

Arachnoid cysts are fluid-filled cyst contained within the arachnoid space. Large cysts may cause intracranial hypertension and require neurosurgical treatment. However, a normal intellectual development in the range of 80-90% is reported by most series.

2.Pregnancy at the cost of severe Ovarian hyperstimulation syndrome

In private practice the pregnancy is the ultimate target of any fertility center, that is true with us too. To place more embryos and to get good blastocyst, there is always an effort to harvest more eggs . This leads to use of more dose of Gonadotropins.

We have experienced a severe variety of ovarian hyper stimulation first time in our carrier and we wish to share with you.

P.P. a 33 yrs lady with tubal cause of infertility came to us for IVF. She was average built and the periods were regular. Husband’s semen was ok.

We took her in long protocol and after downregulation we started 300 IU of uFSH daily . After 3 days FSH was replaced by uHMG 300. ( We usually use this dose regularly above 30 females)On fifth day there were multiple follicles in each ovary and the dose of HMG was reduced to 150 IU and on seventh day it was reduced to 75 IU.

Egg retrieval was done under propofol and 20 oocytes were retrieved. Luteal phase support was with vaginal progesterone 600 mg daily

IV Hexaethyl starch was given for hemodilution . On day 5 ,three blastocysts with good Inner cell mass were transferred atraumatically . On that day ovaries were enlarged but very slight fluid in POD.

On third day of embryo transfer, she came back to us with abdominal swelling and within two days she developed dyspnea . On examination, there was gross ascites and pleural effusion. She was admitted in Intensive Care Unit and her hematocrit went upto 53%          ( normally it is 35%).We gave her  IV starch and albumin alternately each day . We did abdominal paracentesis 6 times with two to two and half liters of fluid each time. We did thoracic paracentesis twice and large amount of fluid came out. We maintained her input output strictly. She was given Tab. Cabergolin 0.5 mg per day, from day 3 of embryo transfer, for ten days. She was continuous on nasal oxygen to maintain the SpO2 above 95%.Subcutaneous low molecular weight heparin started when hematocrit crossed 41 %. It took 9 days to tackle  abdominal and pleural fluid to settle. Her hematocrit went down gradually. Her pregnancy test ( serum beta-hCG ) was positive which she is continuing.

To sum up , we should always be aware about this dreaded complication. It is better to use less amount of gonadotropins, so that we get 9-10 oocyte. It is better to avoid hCG trigger and abandon the cycle ( which needs great courage ). It is better to avoid embryo transfer in hyperstimulated cycle and do the embryo freezing and do the embryo transfer in next unstimulated cycle. It is fare to decide the starting dose do gonadotropin with antral follicle count and not by age alone.