 Hello everyone, I am Dr. Nithya Supadhyay, third year junior resident from Department of Rhetordiagnosis Institute of Post-Referred Medical Education and Research, Kolkata. I am going to present a rare case of proof syndrome under the guidance of Dr. Tapanthi Barsar and I am very thankful to 22nd MRI teaching course from Indian neurologist for giving me this opportunity to present this rare disease overview of my case report. Proof syndrome is a rare congenital disorder that comprises of vascular malformation with the electronical dysregulated at the post tissue and skeletal anomalies. It is a newly described rare overworld disorder with serious morbidity. It is associated with three different miscellaneous mutations, PIK3C agent and part of PIK3CL related over the spectrum that is for TROPS. The proof syndrome has emerged as an uncommon yet distinct clinical entity with some phenotypic variations. Its diagnosis is usually from botanist, truncalist, spinal and foot anomalies clinical and radiological studies. There are only fewer than 200 cases have been identified so far worldwide as per post and children's hospital website. I have attached a screenshot of the website here. Now introduction. Proof syndrome is an acronym for a rare condition comprising of congenital lipometo super growth, vascular malformation, epidermal nevel, skeletal and spinal anomalies. Initially it was termed as Proof but now S have been added with the S emphasizing the skeletal abnormalities associated with the condition. It is now preferred. The long term picture for a child with Proofs depends on age and how serious symptoms are. Associated findings may include truncal subcutaneous fatty overgrowth, wide feet and hands, sandal gap deformity, macro ductile scoliosis, enlarged peripheral nerves, CNS manifestation including neuronal migration defects, ME-migalencephaly, ventricleomegaly, disgenesis of corpus callosum, tethered spinal cord and neural tube defects. It is associated with three different missions mutation in PIK3CG and part of PIK3CL related overgrowth spectrum Proof disorders include Proteus syndrome, Proof syndrome, fibroidic pose, hyperplasia or overgrowth, hemihyperplasia, multiple lipometoces, fibroidic pose infiltrating lipometoces, facial infiltrative lipometoces, megalencephaly, capillary malformation, dysplastic megalencephaly, clipple trinone syndrome. These disorders occur sporadically and comprise malformation of various tissue with asymmetrical overgrowth. Difficult diagnosis, varied symptom and continuous disfiguring overgrowth require concerted multidisciplinary approach in diagnosis and management. Role of radiology especially MRI is very vital in evaluation of such cases. I am presenting here a case report of a one-year-old main child who presented to us with epidermal nevus since birth and trunk, unilateral limb hypertrophies, indebtably and multiple asymmetrical lumpy swelling over face axilla and trunk. Now the actual case report, this one-year-old boy presented to us with the following features, epidermal nevus over trunk since birth, multiple soft consistency, progressive swelling over trunk, axilla and face, indebtably of the left, second and third toe with macro-debtably of multiple toes and fingers, asymmetric limb hypertrophy with discrepancy in length of both lower lip. He was the first child born after an uneventful pregnancy to otherwise healthy, non-consanguine parents. His family history was unremarkable. The child had a delay in motor milestones. However, he had normal mental developmental milestones. His 2D eukaryography report did not reveal any obvious abnormality. We evaluated this patient with MRI, USG and X-ray. MRI of anterior abdominal wall and axilla ribbons, large T2 and STER, hyper intense serpiginous and low belated alters in an intensity area without diffusion restriction, and subcutaneous plane in left axilla, anterior and lateral chest wall and abdominal wall, extending clearly up to a total second left thigh. Hypertrofit subcutaneous fat also noted in left anterior abdominal wall. MRI brain did not show any obvious abnormality except lipometer swelling over the face. These findings were also corroborated with ultrasoundography. X-ray of left foot ribbed macrotectile with syndectyle of second and third toe. The child displayed characteristic feature of flow syndrome and could be distinguished from other disorders of process spectrum based on clinical and radiological features in absence of genetic study. You can see here there are various epidermal nevies over the trunk. T2 MRI sequence, so hyper intense serpiginous and low belated alters in an intensity area without diffusion restriction, in subcutaneous plane in left anterior abdominal wall, suggestive of flow vascular malformations. Multi-septated T2 hyper intense cystic lesions noted in right macular region, left lateral chest wall. The same has been illustrated on USG here. The cystic spaces are not taking up color on Doppler study. These features are likely a venolympathic malformation. Soft tissue consistency swelling in right cheek region which shows T1 and T2 hyper intensity in subcutaneous plane on sagittal and axial sections of MR. USG also shows paticogenicity growth in subcutaneous plane which is suggestive of lipometous overgrowth. Left foot shows macrotectyle with syndectyle of second and third toe. Here macrotectyle also noted in right grade toe, left index and middle fingers. X-ray of left foot in AP projection shows marked hypertrophy, macrotectyle with syndectyle of second and third toe with associated enlargement of phalanges. Now discussing the diagnosis of proof syndrome is based on clinical and radiological features of cutaneous vascular truncal spinal and limb anomalies and needs differentiation from other process spectrum diseases. MRI is an excellent tool for characterization of lesion in adjunct with the USG especially in children where radiation exposure is a concern. MRI is particularly useful for delineating the extent of deformities, their management and assessing long-term prognosis. Classic presentation of this disease is a progressive kind of disease with clinical features described previously that appears with child development and development, epidermal knee, fatigue tissue, proliferation and skeletal anomalies. These children subsequently will grow into adults with variable limitation in everyday life. Severe neonatal presentation could have worse spectrum of malformation with a lower chance of survival premature death by pulmonary failure, secondary to unmanageable intrathoracic masses, pulmonary embolism and intralisthenic bleeding or fatal infections. There is still a limited amount of literature regarding management of the syndrome. This clinician suspecting proof syndrome should go through a thorough radiological and clinical examination of the patient and if required should be referred for genetic testing to confirm PIK3 CS somatic variant to allow for further research into this rare condition. Conclusion, proof syndrome is extremely rare and research in this area is largely confined to a very few published literature. Here when we report few of the past cases of proof syndrome from stern part of India, there is no absolute cure for proof syndrome and management is timely geared towards improving the quality of life. These are my references. Thank you.