A radio-anatomical study of median arcuate ligament syndrome: unveiling the morphology and morphometry of median arcuate ligament, celiac trunk, and superior mesenteric artery.

PURPOSE: In the context of medical literature, a significant lacuna exists in understanding median arcuate ligament syndrome (MALS). While clinical aspects are well documented, literature lacks a robust exploration of the anatomical relationship between the celiac trunk and the median arcuate ligament (MAL). METHODS: Morphometric parameters, including the vertebral level of MAL origin, MAL thickness, celiac trunk (CeT) origin level, diameter, and distances between CeT/Superior Mesenteric Artery (SMA) and the MAL center were observed on 250 CT angiograms. Cadavers (n = 11) were dissected to examine the same parameters and histo-morphological examination of MAL tissue was done. RESULTS: Radiological findings established average MAL thickness of 7.79 ± 2.58 mm. The celiac trunk typically originated at T12. The average distance between the celiac trunk and the MAL center was 1.32 ± 2.04 mm. The angle of the celiac trunk to the abdominal aorta was primarily obtuse. The average celiac trunk diameter was 5.53 ± 1.33 mm. Histological examinations revealed a diverse MAL composition, indicating variable mechanical properties. CONCLUSION: This study provides comprehensive morphometric data on the anatomical relationship between the MAL and the celiac trunk. In contrast to available literature which says the average MAL thickness of > 4 mm is an indicator of increased thickness, we observed much higher average thickness in the studied population. The findings contribute to a better understanding of normal anatomical variations which can serve as reference values for accurate radiological diagnosis of MALS. The histological examination revealed the heterogeneous nature of the MAL tissue composition, suggesting variable mechanical properties and functions in different regions.

Case report of a rare purine synthesis disorder due to 5-aminoimidazole-4-carboxamide ribonucleotide formyltransferase (AICAR) deficiency.

BACKGROUND: AICA (5-aminoimidazole-4-carboxamide) ribosiduria is an inborn error in purine biosynthesis caused due to biallelic pathogenic variants in the 5-aminoimidazole-4-carboxamide ribonucleotide-formyltransferase/imp cyclohydrolase (ATIC) gene located on chromosome 2q35. ATIC codes for a bifunctional enzyme, AICAR transformylase and inosine monophosphate (IMP) cyclohydrolase, which catalyse the last two steps of de novo purine synthesis. This disorder has been previously reported in only 4 cases worldwide, and herein, we report the first from India. CASE REPORT: The proband presented with global developmental delay, developmental hip dysplasia (DDH), acyanotic heart disease and nystagmoid eye movements. Whole exome sequencing (WES) identified compound heterozygous pathogenic variants in the ATIC. A novel splice site variant; c.1321-2A > G and a previously reported missense variant; c.1277A > G (p.Lys426Arg) were identified. Segregation analysis of parents showed the father to be a heterozygous carrier for the splice site variant and the mother, a heterozygous carrier for the missense variant. CONCLUSION: This case of a rare genetic disorder of purine biosynthesis of ATIC deficiency is the first case reported from India. Early diagnosis lead to early interventional therapy and genetic counselling.

Clinical anatomy of the inferior epigastric artery with special relevance to invasive procedures of the anterior abdominal wall.

INTRODUCTION: Injury to the inferior epigastric artery (IEA) has been reported following lower abdominal wall surgical incisions, abdominal peritoneocentesis and trocar placements at laparoscopic port sites, resulting in the formation of abdominal wall haematomas that may expand considerably due to lack of tissue resistance. The aim of this study was to localise its course in relation to standard anatomic landmarks and suggest safe areas for performance of invasive procedures. MATERIALS AND METHODS: Sixty IEAs of 30 adult cadavers (male = 19; female = 11) were dissected and the course of the IEA noted in relation to the mid-inguinal point, anterior superior iliac spine (ASIS) and umbilicus. RESULTS: The mean distance of the IEA from the midline was 4.45 ± 1.42 cm at the level of the mid-inguinal point, 4.10 ± 1.15 cm at the level of ASIS and 4.49 ± 1.15 cm at the level of umbilicus. There was an average of 3.3 branches per IEA with more branches arising from its lateral aspect. The IEA was situated within one-third (32%) of the distance between the midline and the sagittal plane through ASIS at all levels. CONCLUSION: To avoid injury to IEA, trocars can be safely inserted 5.5 cm [mean + 1 standard deviation (SD)] away from the midline (or) slightly more than one-third of the distance between the midline and a sagittal plane running through ASIS. These findings may be useful not only for laparoscopic procedures but also for image-guided biopsy, abdominal paracentesis, and placement of abdominal drains.

Topography of inferior epigastric artery relevant to laparoscopy: a CT angiographic study.

PURPOSE: The incidence of inferior epigastric artery (IEA) injury is 0.2-2 %. The aim of this study was to trace the position and course of the inferior epigastric artery in the anterior abdominal wall above the inguinal ligament at three important landmarks, i.e., at the mid-inguinal point, Anterior Superior Iliac Spine (ASIS) and umbilicus in abdominal CT Angiograms. The study also correlates the relationship of body build and the position of the inferior epigastric artery. METHODS: In 50 CT Abdominal angiograms, the course of the inferior epigastric artery was traced and distance between the artery and midline was measured at the above landmarks using measurement tool on the picture archival and communication system. The measurements were analyzed using SPSS version 16 and expressed as mean and standard deviation. Mann-Whitney test was used to compare the mean values and ratios in males and females. Linear regression was done to derive formulas by which the position of the inferior epigastric artery could be found. RESULTS: The mean distance of the inferior epigastric artery from the midline was 5.17 ± 0.93 cm at the level of mid-inguinal point, 4.57 ± 1.05 cm at the level of ASIS and 5.27 ± 1.17 cm at the level of umbilicus. There was a definitive predictive pattern in the course of the artery as seen in correlation and regression analysis. CONCLUSION: The security distance for safe trocar placement was 6 cm at the level of ASIS and 9 cm at the level of umbilicus. Preoperative IEA assessment is helpful in reducing injuries to IEA.