Dermato-Radiological Evaluation of Congenital Limb Overgrowth Vascular Syndromes.

International Society for the Study of Vascular Anomalies classification defines Congenital Limb Overgrowth Vascular Syndromes (CLOS) as a subset of vascular syndromes with other abnormalities that present with unilateral limb overgrowth. It includes Klippel-Trenaunay Syndrome, Parkes-Weber Syndrome, CLOVES (Congenital Lipomatous Overgrowth, Vascular Malformations, Epidermal Nevi, Spinal/Skeletal Anomalies/Scoliosis) Syndrome, Proteus Syndrome, PTEN Hamartomatous Syndrome, and Fibroadipose Vascular Anomaly. Due to their rare and complex nature, a multidisciplinary approach to diagnosis and treatment is required. A thorough clinical and radiological workup can go miles in reflecting on the patient's outcome. Here we report five cases of CLOS with their detailed dermato-radiological profiles.

A Rare Variant of Zuckerkandl Tubercle: Thyroid Ring.

Zuckerkandl tubercles are posteromedial projections of normal thyroid gland with relations with important neck structures. In our case, we reported "Thyroid ring", that is a ring configuration of the thyroid gland encasing the trachea and esophagus, without compression symptoms. Ring was formed by the elongated zuckerkandl tubercles, passing through the column between the esophagus and vertebral body and abutting each other at left posterolateral border of esophagus. Reporting of these relations and variations is important as it helps the operating surgeon in planning the approach to the procedure. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-04079-4.

Contrast Induced Sialadenitis with Low Volume Non-ionic Contrast in a Patient with Cerebral Venous Sinus Thrombosis.

Background: Contrast Induced Sialadenitis or Iodide Mumps refers to non-suppurative inflammation of salivary glands following intravenous iodinated contrast administration. It is a rare adverse effect of iodinated contrast with only a few cases reported worldwide. It is hypothesized to be an idiosyncratic reaction due to toxic accumulation of iodine in salivary glands. Case Report: We report a case of a 40-year-old female patient who underwent CECT brain after intravenous injection of 40ml of non-ionic iodinated contrast and developed symmetric painless swelling in bilateral submandibular triangles within five hours of contrast administration. Ultrasound with color doppler and MR imaging was performed which confirmed the diagnosis of contrast induced sialadenitis. Sialadenitis was managed conservatively and resolved slowly over eight days. Conclusion: Though it is a rare self-limiting adverse event of iodinated contrast, it must be known to the radiologist as well as the clinician to avoid unnecessary work up and manage the patient better. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03575-x.

Unusual Presentation of Paraosteal Lipoma in Skull.

Paraosteal lipomas are rare benign neoplasm composed of mature adipose tissue with underlying bone changes. They are extremely rare to occur in head and neck region. We present a case of a 40-year-old male with a paraosteal lipoma of occipital region. The diagnosis of the lesion was reached by clinical examination and confirmed on computed tomography (CT) and Magnetic resonance imaging (MRI). Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-023-03494-x.

Evaluation of Joint Formation in Coronoid Process Hyperplasia (Jacob's Disease) on CT and MR Imaging.

Background: Jacob's disease is a rare pathology characterized by elongation/ enlargement of coronoid process of mandible with formation of pseudoarticulation with zygomatic arch. It presents clinically as restricted mouth opening and is often misdiagnosed as temporomandibular joint pathology. Case Presentation: We performed cross sectional imaging and evaluation of Jacob's disease in a 14-year-old girl with restricted mouth opening, CT images including Multiplanar Reconstruction and Volume Rendered Technique revealed enlarged left coronoid process of mandible while open mouth CT images and MR images concluded the presence of joint between enlarged coronoid process and zygomatic arch. Conclusions: Coronoid process hypertrophy and Jacob's disease are important differentials which should be considered in evaluation of restricted mouth opening. We propose that apart from CT, MR imaging should be considered in such cases for better pre-operative evaluation of joint formation. Supplementary Information: The online version contains supplementary material available at 10.1007/s12070-022-03323-7.

Chiari III malformation with defect in Liliequist membrane on MR imaging.

The Liliequist membrane is a radiologically neglected structure, with routine evaluation only carried out in pre-operative and post-operative cases of third ventriculostomy. We report two cases of Chiari III malformation in two unrelated females with similar findings on magnetic resonance imaging study including occipital and low cervical encephalocele, hydrocephalus, and segmentation anomalies in cervical spine. Along with these findings, we report a flow void on T2-weighted images observed in both cases across the site of Liliequist membrane between interpeduncular and chiasmatic cistern. Our findings of CSF flow across the Liliequist membrane may represent spontaneous third ventriculostomy or another congenital defect in the myriad of anomalies seen in cases of Chiari III malformation.

Intramedullary Teratoma of Spine in an Adult Patient.

Spinal teratomas are rare spinal tumors. Most of these present in children. We present the imaging findings of a spinal teratoma that was not symptomatic until adulthood. Teaching point: Congenital spinal tumors may occasionally present for the first time in adulthood, and radiologists need to be familiar with the imaging findings.

Acardius acephalus with spontaneous umbilical cord occlusion: Reporting a rare case.

Twin reversed arterial perfusion syndrome is a rare obstetric condition that occurs in monochorionic twin pregnancies, resulting in coexistence of a normal "pump" twin and an acardiac twin. The acardiac twin is dependent upon the normal twin to provide circulation by means of vascular anastomosis, thereby putting the pump fetus at risk of high output cardiac failure. Overall only 50% of pump twins survive. Mortality for acardiac twin is 100%. We present a case of 26-year-old primigravida female presenting with 8 months of amenorrhea with unsure LMP. Ultrasonography followed by fetal MRI was carried out which revealed acardius acephalus twin with absence of blood flow in umbilical vessels. Pump twin had multicystic dysplastic left kidney with single umbilical artery. Following delivery, the pump twin survived well and the deformed fetus showed features of twin reversed arterial perfusion syndrome.