Cadaveric Study of Varoations in Branching Pattern of Splenic Artery.

Introduction: Splenic artery was previously called as Lineal artery. Splenic artery is the largest branch of the celiac trunk and is the most tortuous artery in the body. Splenic artery mainly supplies spleen and gives off branches to the stomach and the pancreas. Splenic artery divides into terminal branches before entering into the hilum of the spleen which may be Magistral or Distributed type. Sometimes it may pass through the hilum without dividing and supplies the spleen. Materials and methods: The study was done on 50 embalmed cadavers during routine dissection practices for undergraduates in the dissection hall of Gandhi Medical College, Secunderabad, and from the Department of Anatomy Osmania medical college, Hyderabad, during the period of 3years.The variations in the branching pattern of the splenic artery was observed and photographed. The prime objective of the study is to compare the prevalence of variations in the branching pattern of splenic artery. Results and conclusion: The variations in the present study were almost correlated with the available literature. In some instances the left gastro epiploic artery, the posterior gastric artery and the accessory left gastric artery took origin from the interior of spleen. so care should be taken during splenectomy. Hence, the arterial blood supply of spleen is so varied that no two vascular patterns are ever the same.

Comprehensive Study of Neural Tube Defects in 1000 Foetuses with Clinical Spectrum.

Introduction: A variety of malformations are included under the description of Neural tube defects (NTDs). These are abnormalities of the embryonic neuralization process. The congenital malformations of human structure and are of great interest to anatomists, obstetricians, pediatricians and radiologists. NTDs are among the commonest and most severe disorders, affecting 0.5-2 per 1000 established pregnancies, and are second commonest group of birth defects, after congenital heart defects. A valuable contribution of this study, the neural tube defects aimed at clinical methods and refined for the prenatal diagnosis in utero. Materials and Methods: This comprehensive study was undertaken to know the incidence of detail knowledge of neural tube defects in KIMS Narketpally and KAMS & RC Hyderabad, among 1000 births during the period of two years. We found seven fetuses with neural tube defects involving brain and spinal cord. A detailed study was done emphasizing on embryology and genetic and non-genetic concepts. Results & Conclusion: The seven fetuses were stillbirths and aborted babies between 20 to 40 weeks, presented with neural tube defects (0.7%). Five fetuses were females and two fetuses were males. The spinal defects were 0.4%, cranial defects 0.2% and complete neural tube defects is 0.1%. This review article discusses the classification, clinical research and epidemiological understanding of NTDs and correlated with the available literatures.

Study of Morphological Variations of 50 Placentae with Umbilical Cords and its Developmental Relevance.

Introduction: The word “Placenta” is a Latin word and the Greek equivalent word is “Plakons” which means “Flat cake on a plate”. The placenta is a complex multifunctional organ. It provides nutrition, gas exchange, waste removal, endocrine function and immune support. Placenta is a special circulating system to the developing foetus. Being an organ of vital importance for continuation of pregnancy and foetal nutrition it has evolved great interest among the anatomists, embryologists, pathologists and obstetricians. Materials and Methods: The study was done in 50 placentae which were collected from the department of Obstetrics and Gynaecology in collaboration with the department of Anatomy, Kamineni Institute of Medical Sciences, Narketpally, Nalgonda, Telangana, during the period of 2 years. The morphological variations of placenta, the size, shape, weight and attachment of umbilical cord with its blood vessels were observed, recorded and photographed. The prime objective of the study is to compare and evaluate the morphological alterations of placenta and umbilical cord in pregnancy. Results and Conclusion: In the current study, the majority of the placentae showed round shape, few placentae with oval and irregular and with an accessory lobe in single placenta. The current study also includes variations in insertion of umbilical cords was eccentric in majority, central, marginal and velamentous in a few. Pregnancy induced hypertension significantly affects the placenta by reducing weight and it does not have any significant effect on the shape of placenta, umbilical cord insertion and number of cotyledons on maternal surface. The placenta is the most accurate record of the infants prenatal experience.

Lithopaedion.

A lithopaedion or lithopedion is ancient Greek word meaning a stone baby. It is a rare phenomenon which occurs most commonly when a fetus dies during an ectopic pregnancy or during an abdominal pregnancy. The chance of abdominal pregnancy is one in 11,000 pregnancies. If the dead fetus is too large to be re-absorbed by the mother’s body it becomes a foreign body to the mother’s immune system. To protect from possible infection, the mother’s body will encase the fetus in a calciferous substance. The fetus is gradually mummified becoming a stone baby. Lithopedion may occur from 14 weeks’ gestation to full term. It is not unusual for a stone baby to remain undiagnosed for decades and found incidentally when taking plain x-rays films for various other investigations. This paper describes the known case of this phenomenon. The present case was about a 70 year old post-menopausal woman attended the Gynecology OPD with the complaints of mass per vaginum since 5 years. X-ray and Ultrasound scan of abdomen and pelvis revealed a lithopaedion.

Twin Reversed Arterial Perfusion Sequence (TRAP Sequence). The Acardiac / Acephalic Twin.

Twin-Reversed Arterial Perfusion (TRAP sequence) is a rare complication of monochorionic twins (MC, twins sharing one placenta). TRAP sequence is known as acardius or chorioangiopagus parasiticus. It occurs in 1% of monochorionic twin pregnancies and in 1 in 35,000 pregnancies. The risk of recurrence was estimated 1:10,000. TRAP sequence is characterized by a structurally normal pump twin perfusing an anomalous twin. In TRAP syndrome, there is mortality and deformities in both twins. The acardiac/acephalic twin is a parasitic twin that fails to develop a head, arms and a heart. The parasitic twin is with or without legs, receives its blood supply from the host twin (Pump twin). In TRAP sequence, one twin is usually developmentally normal (pump twin) and the other twin has a serious condition, either missing a heart (acardiac) or a head (acephalic) or both, that prevents it from surviving on its own. The acardiac/acephalic twin receives all of its blood from the normal or “pump” twin. The abnormal fetus does not have a heart; all of its blood must come from the normal twin. And it is kept alive by the blood which is pumped from the normal twin through the placenta. The term “reversed perfusion” is used to describe this scenario because blood enters the acardiac/acephalic twin through reversed flow through its umbilical artery and exits through the umbilical vein, which is opposite to the normal blood supply of the fetus.The acardiac twin loses direct vascular connection with the placental villi and receives its entire blood supply from the pump twin [1,2,3,4]. Proper timing of the delivery is of prime importance for the survival of the normal fetus. The emphasis is placed on close sonographic monitoring from early antenatal diagnosis. The mortality of the acardiac twin is 100%, and the perinatal mortality of the pump twin is reported to be around 50%. The mortality rate of the pump twin appears to correlate with the size of the acardiac twin and primarily due to congestive heart failure. We present such a case of TRAP sequence because of its rarity. TRAP sequence, was diagnosed by ultrasound at the obstetrics and gynecology department at KAMS & RC (Kamineni) and it was closely followed up until delivery. Clinical presentation, ultrasound findings, outcome and management were reviewed in present article.

Prune belly syndrome (sequenze): a case report.

The Prune Belly syndrome (PBS) also known as Eagle Barret syndrome is a rare disorder. It is an abdominal muscles deficiency syndrome characterized by a Triad syndrome i.e. deficiency of abdominal wall muscles, failure of testicular descent and dilation of the urinary tract. This syndrome has derived its name from the wrinkled prune appearance of the abdominal wall. Prune Belly syndrome is a rare anomaly seen in one in 35,000-50,000 live births. It occurs in all races. Prune Belly syndrome almost exclusively occurs in males (M:F, 20:1). The diagnosis can be made in utero by ultrasonography at 21 weeks of gestation or in the Neonate with characteristic clinical findings. The present case was a dead male fetus of 20 weeks of gestation sent to Anatomy department after Medical termination of pregnancy, due to congenital anomalies identified in routine ultrasound examination during antenatal checkup.

Limb body wall complex or body stalk complex or cyllosomas: a case report.

Limb body wall complex (LBWC) is also called Body stalk complex and Cyllosomas. We present this rare congenital malformation complex highlighting the importance of early sonographic imaging findings in LBWC along with differentiation from other anterior abdominal wall defects. Limb body wall complex / Body stalk anomaly refers to a rare complicated polymalformative fetal malformation syndrome of uncertain etiology and results in head, heart, lung, diaphragm, kidney or gonadal abnormalities. LBWC was first described by Van Allen et al; in (1987). The two of the three following anomalies must be present to establish the diagnosis: 1. Exencephaly / Encephalocele with facial clefts, 2. Thoraco-Abdominoschisis / ventral body wall defects and 3. Limb defects. LBWC arises as a result of early amnion disruptions or error in embryonic development. If all components of the syndrome are present, the condition is lethal. LBWC is invariably fatal and incompatible with life. No case of postnatal survival is reported so far. Serum alpha-fetoprotein measurement and ultrasonography examination is the key to the prenatal diagnosis and followed by medical termination of pregnancy. It presents two distinct phenotypes described by Russo et al (1993) and later Cusi et al in (1996), according to the foetoplacental relationships: 1. Placento-cranial and 2.Placento-abdominal types. Among the 168 live births at S.V.S. Medical College & hospital Mahabubnagar (INDIA) during the period of 2010-2011 we came across an aborted female fetus. It was weighing 1800gms, 30 weeks of gestation diagnosed by antenatal ultrasonography as ventral body wall defect. It was associated with ompholocele, severe scoliosis and limb defects. Its confirmation of the diagnosis of Limb body wall complex with Placento-abdominal type was done by postmortem fetography.

Apert syndrome (Acrocephalosyndactyly): a case report.

Apert syndrome is named for the French physician. Eugene Apert in 1906 described the syndrome acrocephalosyndactylia. It is a rare autosomal dominant disorder characterized by craniosynostosis, craniofacial anomalies, and severe symmetrical syndactyly of the hands and feet (i.e. cutaneous and bony fusion refers to webbing of fingers and toes). Apert syndrome is characterized by the premature fusion of certain skull bones (craniosynostosis). This early fusion prevents the skull from growing normally and affects the shape of the head and face. In addition, a varied number of fingers and toes are fused together (syndactyly). Most cases of Apert syndrome are sporadic, may result from new mutations in the gene. The purpose of this paper is to report a case of Apert syndrome in a female fetus of 30 weeks with asymmetrical skull confirmed by prenatal ultrasonography. Pregnancy was terminated and fetus was submitted for detailed autopsy in anatomy dissection hall. The findings and review of literature were presented in this article.

Cadaveric study of accessory renal arteries and its surgical correlation.

A thorough knowledge of the accessory renal arteries has grown in importance with the increasing number of renal transplants and other uroradiological procedures. The literature indicates that multiple renal arteries are found in 9-75% cases. Normal anatomy describes each kidney receives irrigation from single renal artery which arises from abdominal aorta at the level L1-L2 vertebrae just below the superior mesenteric artery. Renal artery variations include their origin, number and course. The most common is the presence of additional vessels (accessory arteries) arising above the usual trunk is more frequent than one arising below. The accessory renal arteries are always end arteries. The kidneys may receive a single artery although each organ may equally be supplied by as many as six end arteries. The right and left renal arteries may arise from the aorta by a common stem or arise at lower point than usual in which case the kidneys lie below their usual position. There may be several renal arteries on each side or the renal artery may divide close to its origin into several branches. Current literature reports great variability in renal blood supply, the number of renal arteries mentioned being the most frequently found variation. Normal renal arterial information is useful not only for planning and performing of endovascular, laparoscopic uroradiological procedures and renal transplants. In order to facilitate the clinical approaches, we studied renal arterial pattern in 25 formalin fixed cadavers, on 50 kidneys during the period of one year. The purpose of this present study was to establish the incidence of accessory (aberrant) renal arteries in human cadavers and also discuss its surgical correlation during uroradiological procedures and angiographic interventions.

Cerebrotendinous xanthomatosis: need for early diagnosis.

Cerebrotendinous xanthomatosis is a rare autosomal recessive lipid storage disease characterized by widespread tissue deposition of two neutral sterols, cholestanol and cholesterol, resulting in tendinous xanthomas, juvenile cataracts, progressive neurological defects and premature death from arteriosclerosis. The primary biochemical defect is deficiency of hepatic mitochondrial enzyme sterol-27-hydroxylase which catalyses the hydroxylation of cholestanol (5-alpha dehydro derivative of cholesterol) and this deficiency decreases bile acid synthesis. Substantial elevation of serum cholestanol and urinary bile alcohols with low to normal plasma cholesterol concentration establishes the diagnosis. Cerebrotendinous xanthomatosis is exceptionally rare in the Indian population. We are reporting a woman with this rare disorder, who was on antiepileptic and antipsychotic drugs for a prolonged period and whose original condition went undiagnosed. She presented with xanthomas on the Achilles tendons and the upper end of tibia. She was mentally subnormal and her serum cholestanol level was raised. Her younger sister too was severely affected by this disorder. Early treatment with chenodeoxycholic acid is known to prevent disease progression.