User:Gsjohri
Introduction
[edit]Dr. Girja Shanker Johri MBBS; TDD; DMRE; MS (SURG) presented this specimen of Foetus & Fetu to Department of Pathology of his parent college KG Medical college. This was at the time of winding up from India to USA. Dr Johri is the graduate of KG Medical College 1948-49 batch. He was selected in class one of the UP Medical Services after Graduation. He is a Senior Member of Association of Surgeons of India. He served for almost 34 years in different capacities & retired in year 84-85 as Director Medical & Health; World Bank Projects of Utter Pradesh India. He had a number of good interesting cases worth reporting in literature to his credit during his career and so is this case so called as Foetus in Fetu.
Actually all the Teratomas Group of Tumors encountered in Pathology are due to the displaced cells of embryonic plate in development of the foetus in intra uterine life. In later life it is seen as a tumor in body containing different type of body tissues or the different part of the body inside when the Operated Tumor specimen of surgery is dissected . These contents differ depending on the type of displaced cell in foetal life that is a ectoderm, endoderm or mesoderm or its combinations So the Surgeons they encounter different tissues like hair nail out of ectoderm , bone out of mesoderm cells etc or are when in combination some body tissue like hand or foot etc. The group of cases where body parts were encountered in tumor were labeled it as foetus in past. In middle of 20th Centuary Nicholson and Willis in their study of teratomas, pointed out that if the tumor presents as a whole or part of the vertebral column, only then are they to be classified as foetus. Invariably all such tumors where the spine formation was there were due to the incorporated mono ovular or bipolar twin in intra Uterine development of life appearing with a distorted apperence due to mal development. This group of tumor of incorporated twin is an interesting & rare entity in the literature. This reported case of Dr Johri is of the category. It was encountered in1968 by him when posted in Bareilly UP India during Surgical operation of a baby boy of 20 months who had symptoms of Intestinal Obstruction. This rare entity reported by him was then the thirteenth case when operated of all those reported earlier in the series in International Literature since 1800. This specimen of this thirteenth case also happened to be largest in size & weight of the series . The Host of the specimen from whom it was taken out was also oldest in age in the series. The whole series since 1800 is shown in the following table with all the details.
This case was operated by him when posted in Bareilly in year 1968. It is an interesting entity and it was the thirteenth case in the series of International Literature since 1800. His specimen was largest in size & weight in the whole of the series when operated. The Host from whom it was taken out was also oldest in age in the series.
Table of Cases From 1800 to 1968
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* = Museum Specimen restudy by Lords by radiological examination / ** = Specimen destroyed in Bombing of London*** = Republished by T. Fuji Kora & W Hunter | |||||||||||
Case History
[edit]Munna Lal s/o Kishori Lal, aged 20 months, resident of Badaun 30 miles off Bareilly, U.P. India was brought with signs of intestinal obstruction in out door of the Hospital. The parents gave a history of growing lump in the abdomen from the age of 6 months and lately having attacks of pain and vomiting with loss of weight. The patient was admitted on Saturday, December 16, 1967. On examination it was found that the abdomen was bloated uniformly with occasional visible peristalsis with a palpable intra abdominal lump ovoid in shape in the area of attachment of mesentery more to the right, slightly movable in oblique directions. The percussion note was dull. Heart, lungs, liver and spleen were normal. His condition improved with conservative line of treatment of obstruction and was put to investigations for further action. All investigation came out to be normal except low RBC & Hb %. The X-ray of abdomen dated Tuesday, December 26, 1967 showed an image of a fetus inside with spine on left and the pelvis hip joint inferiorly with attached long bones. It was decided that the lump was a fetus. Thus an abdomen exploration was decided. On Tuesday, January 30, 1968 abdomen was opened by a right oblique incision (Picture 8). The lump was retroperitoneal at the root of mesentery size 9x4 inches with coils of intestines stretched out on it. Sup. Mesenteric artery was felt posterior to the lump. The lump could be moved and seem to be attached to porta hepatis. A pull on the lump was dragging the liver also. Attempt to decapsulate the lump with a small cut on the avascular area of mesentery failed as it closely adhered to the vascular capsule. Retro peritoneal approach was not possible too and so the lump was removed with posterior peritoneum resecting of a portion of stretched small intestines separating posteriorly the Superior Mesenteric artery. A few slender branches of the artery were ligated also. An end to end anastmosis was done and continuity of the bowl was restored after the tumor was delivered. A cord like strand, avascular to porta hepatis also had to be cut before the delivery of the Tumor. The small bowels were found full of round worms which were cleared before bowel anastomosis. The gap of peritoneum was closed by stitching the free ends and the abdomen was closed in layers. Post operative was an anxious period for few days because of distension & there after was uneventful but was kept in isolation to protect him from visitors.
Gross Anatomy Of The Specimen
[edit]The specimen is ovoid in shape completely covered by capsule all around (Picture2). It is broader at one end than the other with rough posterior surface & smooth anterior, covered with peritoneum with intestines hanging with mesentery almost measuring 28 cms. on its free border running across this surface. The opening of the bowel shows two round worms cut through (Picture 3). A small cord like structure on posterior Surface which was severed during surgery was hanging. Two small arteries which were ligated during surgery while separating the mass is seen hanging and merging in the capsule. The capsule was opened from posterior lateral side as anteriorly it was firmly adherent. As soon as I opened the capsule I entered a cavity from where two feet of lower extremity came in view. In the cavity sebaceous material like Vernix caseosa was discovered. The opening was further explored and few stray strands from the capsule were found entering the mass, one was very prominent and was entering the left great toe. Further reflection of the capsule revealed a fully formed Foetu as shown in the picture 5 and 6. An Umbilical Cord seven centimeters in length was also seen emerging from abdominal wall and merging in to the inner side of the capsule (Picture 5).
Gross Anatomy Of The Foetus
[edit]
The specimen weighs 1500 grams and measures 42 centimeters from fore head to the toe almost in womb position completely flexed with extended legs. In oblique view it gives a look of a fully formed foetus about six months of intra uterine life (Picture 6). The whole body is fully covered with skin and fine hair. There was a greasy Vernix Caseosa type material all over. Head is well formed. The girth of head is 28 cms. It is cystic in consistency. Obviously there is no bone formation in it. Outer capsule is badly adherent to scalp specially on the left side. Head is covered with hair. Occipital Protuberance is not very prominent. Neck is short and gradually extending on to the well formed back of the body. Face of foetus is hidden behind the two well formed extended thighs & legs. On inspection by separating the two feet, it is found that fore-head is well formed. There is no formation of face including eyes or ears. A small process found projecting in place of nose with two grooves extending laterally from it and one groove extending down from it. It seems to be an incomplete attempt of Maxillary and Mandibular arches in formation of face. The place of Jaw is confluent with front of the chest.
The back is well formed with posterior central groove fagging down below in to natal groove. Shoulder formation is not prominent but the fact is obviously not felt because the inferior extremity is so well developed & covers the whole body hiding it completely. Occiput to coccyx measures 18 cms. Superior extremity seen as two bunds measuring 2 cms. on right and 1 cm. on left side. Demarcation of fingers are not obvious.
The abdominal wall is well formed with distinct umblical cord attachment connecting the foetus to capsule. This part is also hidden between abdominal wall and acutely flexed thighs on it. A separate string is seen extending from the capsule near the umblical cord attachment to face near the projecting mandibular arch described above.
Buttocks are well formed 2.9 cms on either side from the anus. Anus is well formed but not patent. Penis is formed size 7 mm in size. Scrotal fold is present united in midline but no testis in them. The thighs acutely flexed on abdomen measuring about 9 cms. in length and in width, knees are not well formed and is in extended position. Legs are well formed each measuring 7 cms. in length and width. The two feet are well formed. Specially the left foot, which is having deformity. All the toes and nails on them are well formed and clearly seen. The greater toe of Telepes Calcanium left foot attached with a tag to outer capsule is magalic along with better development of whole of the foot.
A few stray string like structures is found connecting the outer capsule to the inner mass. One of them is very prominent and is entering the left great toe.
Internal Anatomy Of Foetus
[edit]The specimen was put to detailed study by dissection and histology. Since very few cases have been reported in the literature and the pride expressed by different museum abroad in possession of such a specimen, study has been done with an idea of preservation of specimen rather than the study after its destruction. As noted from the details of different cases, this specimen is quite a large, well-formed one and one of its kind in the whole series in international literature. Keeping in view the above facts, the specimen was dissected from ventral side and the internal development has been studied macroscopically and microscopically.
The abdomen and chest cavity was opened by longitudinal incision. The thorax was not well developed without any demarcation of chest and abdomen, as diaphragm could not be traced out. Just from the upper part of the cavity a little tube was found hanging from dorsum by fold of mesentery. The middle part of the tube was tending to be in coils, and was attached to anterior abdominal wall near the umbilicus. The small and large bowel could not be demarked out. The total length of the tube is about 18 ½ cms. thick in proximal portion ¼ cm in middle and distal part.
Histological Study
[edit]GUT: Sections were put to histological studies from different portion of the tube and all of the three sections showed similar picture of gut wall with sub mucus and muscular layers but no mucosal lining inside. There was a diffuse infiltration of round cells in all of them.
COVERING: Section hardly showed any tissue
CAPSULE: Capsule which has already been described above was also put to a microscopic study. It consisted of fibro collagenus tissue enclosing number of thin walled blood vessels 9 Sec. No. 4493. Acc. No. 3178.
SKIN: Skin by naked appearance is just normal one with distribution as described above. A piece of skin from abdomen was put to histological examination. It showed all appendages, hair follicles sebaceous gland sweat glands etc. The epidermis in the section is thin and keratinized.
There was no trace of liver or spleen. Heart and lungs could not be traced out. A small Bulbous shaped tissue was seen on both sides in the upper part of the cavity at the beginning of the tube, this might be the representation of the lung buds but could not be confirmed by histology.
BLADDER: Tissue was taken from the bladder area where demarcation was not complete. It showed fibrocollagenus tissue with smooth muscle fibers. There was diffuse infiteration of round cells, but there was no mucosal lining seen inside.
BONE: A piece of bone was put to histological examination. The section showed thin trabeculae separated by marrow tissue with evidence of calcification.
PENIS: One small piece of tissue measuring 1.0 cm x 0.5cmx 0.30 cm was put to a section. It showed lining of stratified epithelium. The sub epithelial connective shows large number of vascular spaces.
GLOBULAR MASS OF FACE: A piece measuring 1cm x 1 cm of the later showed loosely arranged fibrocollagenus tissue lined on one side by stretched out stratified squamous epithelium.
UMBILICAL CORD: The tissue from umbilical cord measuring 5.0 x 0.2 cm on section showed fibrocollagenus tissue with few smooth muscle fibers lined on one side by stretched out stratified squamous epithelium but no blood vessels were seen.
RADIOLOGICAL EXAMINATION OF THE FOETUS:
The foetus with the capsule was put to a radiological examination in antero posterior and lateral views and also with injections of radio opaque material through cord and anus. In AP view a nice estimate of bony development was available. There was no development of skull bones on either sides and also of the sternum. In the vertebral column twelve pieces could be counted with spaces in between, the intervertebral space. The column tapered down below with formation of last four pieces in form of sacrum and Coccyx. A mass is seen projecting in front under the soft tissue shadow of skull with similar shadow on the other side. This corresponds to superior extremity buds with no demarcation of different bones of arm and forearm. The terminal part of this mass is expanded with about small radio opaque shadows for both sides representing the short bones of the hand.
Pelvis is not well formed. But projected mass on both sides from the lower end of vertebral column anteriorly only seems to be the early formation of pelvic bones, where two long femurs are attached in full flexsion. The femurs are well formed and long enough to reach beyond the chest. Tibia fibula bones are not well formed, are short and are in continuation with femur with joint space in between in extended knee position. Foot bones like calcaneum and other short bones like tarsal bones are seen though cannot be recognized by name.
The injection of radio opaque material through anus showed that it is not patent. The injection of radio opaque material through cord also did not show they are patent beyond the abdominal wall.
General Impression And Introduction Of Subject
[edit]The matter is very astonishing to all non medical persons, unbelievable to them till they look at it and convince themselves. For medical profession this is not new but rare & confusing for many with Teratomatus tumors till they go deep in the matter of the subject. I may point out that Teratoma, a tumor, which is formed in body by a displaced multi potent or toti potent cells of embryonic plate is thought to be an attempt of a new foetus formation in the body. The embryonic plate consists of ectoderm, endoderm and mesoderm cells. The content of the tumor depends on predominant type of cell. Thus ectoderm component when are displaced during foetal development give rise to a mass or tumor having its derivatives like hair, nail, teeth etc. It is not unusual to see such entities in surgically removed specimens. There are number of cases where even a hand or other part or any organ is seen in the tumor. These specimens in past were labeled as foetus. But Nicholson and Willis in their study of teratomas, pointed out that if the tumor presents as a whole or part of the vertebral column, only then are they to be classified as foetus because for the normal development of the foetus, it is essential to have basic first structures of the body, the central axis which is followed by secondary influences by which the germ layers are laid out in an orderly way followed by organogenesis subsequently. A deviation from this normal, on other hand, on a displaced multipotent cell with no directive influence always results in a disorderly growth may be with organogenesis but without proper Central axis results in tumors labeled as teratomas. But the category of Foetus in Fortu is of its own kind extremely rare entity in the literature.
Now let us recall the Human Embryology for genesis of twins. The ovum conjugates with sperm to form a zygote in fallopian tube and after this division into two, the cells continues to divide into millions and forms a blastomere with demarketed inner cell mass and then to the stage of morula while traveling down the tube towards the Uterus. The first group of cells to differentiate in morula is trophoectoderm cells and they form the chorionic membrane and placenta, getting embedded in uterus.
The twins are classified as monozygotic in which twins are from a single zygote. The monozygotic twins are identical twins and always have a single sharing placenta. If multiple ovums conjugate separately with separate sperms they form the Dizygotic twins which are dissimilar twins all having separate placentas and different cords.
Monozygotic twins have other varieties too depending on the stage of twinning which are as follows:
- The cells up to early morula are totipotent and by separation can form a twin but then both foetuses will have separate set of membranes and therefore called Monozygotic Dichorial diamiotic twins. In human beings only 5% of the twins are of this group according to Dr Willis but it is common in rats etc.
- In late blastocyst stage to morula the cells of inner cell mass are multipotent type and twinning at this stage will be monochorial and diamniotic. According to Dr Willis 75% of Monozygotic twins are of this group. That means the twins will have same placenta but in their own separate sacs.
- If twining occurs after the formation of amniotic cavity it results in conjoint twins and monsters. We come across foetus with two heads or two bodies joined together and are called monsters.
The foetus in foetu are the twins of verity B i.e. Monozygotic monochorial and diamniotic. The process of inclusion is what one has to understand now, to reach to the final conclusion. In the normal early foetal life at the age of about 10 to 11 weeks of intra uterine life the intestines of the embryo develops out side its body in yolk sac. At about twelve weeks physiologically the intestines are sucked in for the abdominal wall to grow over it. In this process the twin brother of Munna Lal happened to be sucked in also in his abdomen with the intestines, and it became a parasite to his host brother. Due to the depended nutrition and less of space it continued to grow but with much less a pace than his host and as a Tumor in the abdomen of host brother. In this case the tumor continued to grow in his brother’s abdomen in extra uterine life also to a stage when it was large enough and started causing intestinal obstruction of the host and resulted in the parents reporting to the hospital for help.
Summary
[edit]Patient Munna lal was brought to hospital in outdoor on 17 Dec 1967 with acute on chronic obstruction. On investigation he was found to have foetal bones in the lump he was carrying. The diagnosis of Foetus in Foetu was clinched after visualizing the vertebrae in the ski gram. The patient was operated. Tumor in a capsule was retro peritoneal in root of mesentery with intestines stretched over it as in picture. The only way to remove the mass was with peritoneum with stretched bowels measuring about 40 cms on the free end followed by end to end anastomosis. On opening the capsule, a well formed foetus weighing 1500 grams was found inside as in picture.
The foetus was a monozygotic diamniotic twin brother of Patient Munna Lal which had happened to be incorporated in his abdomen at 10 to 12 weeks of intra uterine life on physiological sucking up of intestines developing in yolk sac and there after the abdominal wall formed over it. Thus it became a parasite to his brother and continued to grow on a slower pace due to lack of space and nutrition even in extra uterine life also, to a stage, big enough to cause obstruction. (Monozygotic twins are formed when a fertilized ovum cell divides in two called Zygote and the two cells separately divides into millions to form two foetuses). He was operated and the twin brother in a capsule was removed from his abdomen and preserved. This is a rare entity. This case was 13th in international literature since 1800 A.D. when operated on 26 Dec 1967. For details please go through the main text.
Acknowledgments
[edit]I am grateful to Dr. R.M.L. Mehrotra, Prof. Head of Department Pathology King Georges Medical College for his help and guidance in study of this entity. The histological studies have all been done in Department of Pathology under the supervision of Dr. Mehrotra. He was kind to provide me some important literature too. I am also grateful to Dr. A.C. Dass, Reader Department of Anatomy of King Georges Medical College, Lucknow for providing me a guide line and the literature of concerning anatomy. I am also thankful to Shri V.P.S. Nigam for the Photographic work. I am grateful to Dr. U.C. Gupta for his assistance in operation and postoperative care of the patient. I am thankful to Dr. A.U. Khan Civil Surgeon Bareilly U.P. India for the permission to publish this case.
Bibliography
[edit]Lord Joshephine N. ( 1956) Intra-abdominal foetus in Fetu J. Path. Bact. 72 No. 627-641.
Schoenfeld M. ( 1841) Ann. Gyer, E. Pedcited by Lod Losephine M in above.
Taylor. S. ( 1887) Trans Path. ( Soc. Sen) 38 – 440 case cited by Lord Josephine in above.
Highmore W ( 1815) A case of foetus formed I a young man by cited by Lord Josephine in above.
Young C. W. (1800) Med Clier Trans 1234 case cited by Lord Josephine M. in the above.
Dr P Bose and his exhaustive anatomical and developmental study in the 12th case of the series.