Wednesday, February 24, 2016

BENIGN DYSGERMINOMA

UNPLANNED PREGNANCY WITH LEFT OVARIAN
TUMOUR - DYSGERMINOMA.
One of my regular patients called Wendy aged 26 years came to see me one morning in September 1982 requesting a termination of her pregnancy. At this stage she was 8 weeks pregnant. I had delivered her previous two babies who were aged 4 and 2 without any problems. I am not keen on terminations but I agreed to do this as she was finding it difficult to manage two infants without adding another one. Incidentally and luckily, I had a new Ultrasound machine brought to my office for demonstration. I thought it will be good to use this machine on Wendy. She agreed to it. It confirmed an 8 week pregnancy. I tried not to show her the foetus. But what I saw was a bit worrying. She had a 6 centimetre left ovarian tumour, both solid in some areas and cystic (filled with fluid) in others. It was knobby in appearance on the surface.

A Diagnosis of ovarian Dysgerminoma was made. Dysgerminoma is a germ cell tumour (primitive cells in the ovary from which all parts of the body grow) of the ovary. It accounts for 1 % of all ovarian cancer. It is common in children, adolescent and young women. It seldom occurs after 50 years of age. It seldom produces symptoms in early stage, as in our present case. The diagnosis is often made fortuitously on routine pelvic examination or ultrasound done during pregnancy. They produce certain substances in the body which can be useful in the diagnosis. These are called tumour markers. These would not have been useful in this case as she was already pregnant. The next important step is to know the staging of the tumour. A simple way to understand this is, if it is confined to one ovary or both ovaries, or it has spread to the surface of the ovary peritoneum and distant organs. Like this they are divided into four stages. By ultrasound I could make out that Wendy’s tumour was very early. The other ovary and the uterus were normal there was no fluid in the abdominal cavity. With this in mind I explained the situation to Wendy. I proceeded to operate on her. I terminated her pregnancy and proceeded to a laparatomy. The ovarian tumour was soft and solid. Peritoneum was clean and I washed the peritoneum and collected this fluid for pathology to look for any malignant cells, no lymph nodes were felt and the liver felt normal. Dysgerminoma can be malignant, but in early stages a simple removal of the ovary with the fallopian tube gives excellent results. This procedure is called unilateral salpingo-oophorectomy. The patient was discharged on day 5. The pathology reported it to be Dysgerminoma without any features of concern. The oncologist was of the opinion that no further therapy was required. Her pregnancy test became negative after four weeks. I took her under my care for the next 5 years. In the first year I did an ultrasound every 3 months then every 6 months and then every year. I saw her after 20 years; she was divorced from her first husband and had had 2 more children in the new marriage. Wendy was very lucky that her unplanned pregnancy became a life saver for her.

Fortunately I had a trial Ultrasound Machine which made the early diagnosis of her stage one Dysgerminoma Tumour possible. A simple unilateral salpingo-oophorectomy saved her fertility and her life.

In adults 3 percent of these can be malignant but they respond well to chemotherapy and radiotherapy. 

Wednesday, February 17, 2016

SPLENIC PREGNANCY

A TYPE OF ECTOPIC PREGNANCY

An ectopic pregnancy is a pregnancy which takes place outside the uterus. The incidence of ectopic pregnancy is about 20%.  The fertilized egg usually implants itself in different parts of the fallopian tube, sometimes on the ovaries and rarely on other abdominal organs. The tube and the abdominal organs cannot sustain this pregnancy, this can rupture or burst and cause intractable haemorrhage, which becomes life threatening and requires urgent treatment. When the fertilised egg implants to start with on other abdominal organs it is called primary abdominal pregnancy, rarely the pregnancy is expelled from the fallopian tube and settles down on abdominal organs. Such as, Omentum (It is a sheet of fatty tissue with the abdominal lining called the peritoneum that insulates the abdominal organs, Latin for apron) intestines or liver and continues to grow into a baby, the placenta also forms. This is called a secondary abdominal pregnancy. About 1.3% of ectopic pregnancies are abdominal pregnancies many cases of abdominal pregnancy are reported, few above the age of viability,   some even at full term. The ectopic pregnancy at any time is a life threatening problem. In recent decades it has become very much easier to make a diagnosis with the advent of pregnancy tests, very high quality Ultrasounds and MRI’s. Treatments have simplified as well. An operation can be avoided if the diagnosis is made in time. I always remember when a few friends had gone for a picnic about thirty kilometres from our medical school. One of my friends, whose husband was with us, started to feel severe abdominal pain and felt faint, we rushed back to the hospital having immediately made the diagnosis of an ectopic pregnancy, but we could not save her. This was in 1960.The pregnancy I am talking today about was a primary abdominal pregnancy on the spleen. This is one of the rarest sites for an abdominal ectopic pregnancy. So far, only nine such cases have been recorded in English literature to date. I found another case described from India in 2011 bring the total to 10 and I wonder if I can count my case as number eleven, as we never published this. This was in early1960 when I was an intern.
A woman called Katori aged 18 years was rushed to our emergency room, I was on duty. She was thought to be pregnant. Her period was 6 weeks late. It was about 6 AM. She was extremely; restless I was unable to record her blood pressure. I thought that I could feel a very feeble pulse in the neck. There was no time to do too much. Toad pregnancy which was the only pregnancy test available at this time in the world was not possible because it takes a day to get the results. There was no ultrasound in those days. There is a test called shifting dullness, which was positive, which meant that her belly was full of blood. A diagnosis of ruptured (bleeding ectopic) pregnancy was made. I put her on two intravenous drips, one was a dextran drip which was to improve the blood volume and the other was  called a noradrenalin drip to improve her bold pressure. I got her to the operating theatre quickly which was only a few feet away. I sent for my chief resident and my Professor who happened to be a general surgeon as well. The theatre was ready for the patient to be operated on, all the staff we had were there within fifteen minutes.  We had two units of O negative blood (universal donor) which was also started. We had no proper anaesthetist or a person trained in resuscitation. In those days, one of the resident doctors had to give open ether for anaesthesia. I had to do that on the day. One other resident had gone to the pathology laboratory with three of Katori’s relatives to cross match the blood. In those times we had to cross match the blood ourselves. My heart was in my mouth when I started the open ether, it was not difficult as she was very flat any way.  Katori’s  belly was opened it was full with almost  two litres of blood. This blood was filtered in a very simple way and transfused back to her. She was found to have a ruptured pregnancy on the lower pole of the spleen.  A spleenectomy was performed, which did not take much time. The abdomen was closed. For a few moments the woman seemed to have improved. We could record her blood pressure as 60/40.  One further unit of blood was being cross matched from one of her relatives. I was able to wake her up from the anaesthesia, which was such a relief. I could breathe again.  The ectopic pregnancy was confirmed on histology as few chorionic villi were seen.
She continued to show ups and downs in her condition with noradrenalin dextran glucose and saline drips, but she was not conscious or passing much urine. She finally passed away after twenty four hours probably due to kidney failure. We could not save her. There are many cases from my first two years as resident which I will never forget. This was one of them. It was like a war zone, no equipment, no highly trained personal and only the Professor.

Once upon a time ectopic pregnancies were a major cause of maternal death. 1n 1980-2007 as many as 56.5% per 100,000 live births and has been come down slowly to 0.50% of maternal deaths out of 100,000 live births, in 1980-1984. It was estimated that with current improvements this can decline further to 0.36 % of 100,000 live births. Unfortunately the racial and age disparities, persist. In countries with poor facilities, the deaths will continue. It was very reassuring that out of  the ten Splenic pregnancies reported in English literature there were no deaths. The main reasons are awareness, very early and very reliable pregnancy tests (we have come a long way from the toad test) very good ultrasounds and MRI. 

Wednesday, February 3, 2016

OBSTRUCTED LABOUR

This is a sad story about the failure of a labour to progress. It is not for those, who are faint hearted. Why does the progress of a normal labour get blocked? It is if the baby is too big for the size of the mother, or is it lying in a funny abnormal position; however the most common cause in the developing world is a distorted or small pelvis. This often happens because of poor nutrition from child hood causing problems such as rickets or in adolescent girls resulting in bone deformities called Osteomalacia. This causes not only a deformed pelvis, but deformed bodies. Often culturally more attention is placed to the nutrition of boys than the girls, and if there is not enough to feed everybody the girls suffer. Sometimes the mother is too young and not fully developed. In spite of many efforts by WHO, in some countries the maternal mortality remains as high as 410 deaths per 100,000 births due to obstructed labour and besides maternal deaths this causes long term severe maternal morbidity by way of vesico vaginal fistulas. There are half a million women in the world with fistulas, mainly in Africa and India. The college of obstetricians and gynaecologists is working very hard to prevent them happening by preventing   obstructed labour, better maternal care during labour, and also curing the fistulas in large numbers. The story I am going to tell you in this post is of one such girl.  I will have more stories about obstructed labour in some of my future posts.
This was in 1968, a very young girl named Renu aged 17 was brought to the hospital by her husband and brother on a home bed (CHARPAI).

She was in strong labour for some hours. She had not been able to walk for two years. Her thighs and legs were totally folded over her abdomen. She obviously was pregnant; and according to her mother in law almost full term. She had never seen a doctor or even a midwife. I wondered how the groom married her. He seemed a bit simple, or had she become disabled recently. Her pelvis was so narrow I was unable to insert even one finger and even the rectal examination was not of much help. I wondered how she had even become pregnant. She had to have a caesarean section (C.S) to have this baby delivered. 

There are two types of C.S, a lower uterine section, when a transverse cut is made on the lower part of the uterus, this is a safer C.S, as it is less likely to be torn or as we call it ruptured in the next labour. The other C.S. is called a classical C.S, this is a long cut made in the upper part of the uterus. This is a more risky procedure as it is likely to rupture more often ( 4% to 9% as compared to lower uterine scar which is less than 1%). Once a woman has had a classical scar, she must always have a hospital delivery by C.S.  In this case I had to do to a classical C.S.as I could not reach the lower part of the uterus due to the extremely narrowed pelvis. To add to my sadness when I pulled out the baby it was grossly abnormal weighing only 2.2lbs. It was what we call an anencephaly, this means that a part of the babies brain and skull failed to develop.

This is incompatible with life, so we did not revive the baby. There are many stories for anencephalic babies living for a while. The longest reported baby lived for 3 years. I once worked in an hospital in the UK, the sister in charge of the maternity ward did not allow us to let an anencephalic baby to be treated how we normally would have treated them. This one particular baby lived for 11 days. These days diagnosis of an anencephaly is made early on with an ultrasound, all women are offered an abortion, which most of them have. In 2012 about 208 terminations were performed in England and Wales.  Neural tube defects occur 1 in 1000, in the western world, were as it can be 5 in 1000 in developing worlds. The recurrence rate can be 4-5%, if there is a previous history of having had two such babies the risk raises to 13%.

Main reason for this to happen is poor nutrition and folic acid deficiency. In the western world this had been prevented by generous use of folic acid. In this case we could see the poor nutritional effect on the mother as well as the baby. We had lots of instructions for the family before they went home. Firstly we advised contraception for at least, 5 years. Regular visits to the village health centre, vitamin D, calcium, milk, eggs, and a high protein diet, regular physiotherapy, massage and plenty of sunshine. The next pregnancy must be supervised, and must be by C.S. in a hospital. Renu’s health could be improved if she was taken care of seriously. I found out that all this happened because of ignorance and carelessness rather than poverty. Unfortunately   I was unable to follow her case as I had to leave the country.