ABSTRACT
Perioperative management of a patient with Dandy–Walker malformation (DWM) with tetralogy of Fallot (TOF), patent ductus arteriosus, and pulmonary artery stenosis is a great challenge to the anesthesiologist. Anesthetic management in such patients can trigger tet spells that might rapidly increase intracranial pressure (ICP), conning and even death. The increase in ICP can precipitate tet spells and further brain hypoxia. To avoid an increase in ICP during TOF corrective surgery ventriculo‑peritoneal (VP) shunt should be performed before cardiac surgery. We present the first case report of a 11‑month‑old male baby afflicted with DWM and TOF who underwent successful TOF total corrective surgery and fresh autologous pericardial pulmonary valve conduit implantation under cardiopulmonary bypass after 1 week of VP shunt insertion.
Subject(s)
Anesthesia, General/methods , Cardiopulmonary Bypass/methods , Dandy-Walker Syndrome/epidemiology , Dandy-Walker Syndrome/surgery , Heart Valve Prosthesis Implantation , Humans , Infant , Male , Perioperative Care/methods , Pulmonary Artery/transplantation , Pulmonary Valve/transplantation , Stents , Tetralogy of Fallot/epidemiology , Tetralogy of Fallot/surgery , Transplantation, HomologousABSTRACT
Mayer-Rokitansky-Kuster-Hauser (MRKH) is a malformation complex comprising absent vagina and absent or rudimentary uterus. MRKH syndrome may be attributed to an initial affection of the intermediate mesoderm consequently leading (by the end of the 4th week of fetal life) to an alteration of the blastema of the cervicothoracicsomites and the pronephricducts. These latter subsequently induce the differentiation of the mesonephric and then the Wolffian and Mullerian ducts. There are very sparse such cases reported. We present a case of type II MRKH or Mullerian renal cervical somite association (i.e., Mullerian duct aplasia, renal dysplasia, and cervical somite anomalies).
Subject(s)
46, XX Disorders of Sex Development/epidemiology , Abnormalities, Multiple , Adult , Congenital Abnormalities , Dandy-Walker Syndrome/epidemiology , Dandy-Walker Syndrome/genetics , Female , Humans , Kidney/abnormalities , Kidney Diseases/congenital , Mullerian Ducts/abnormalitiesABSTRACT
Dandy–Walker malformation (DWM) is a rare intracranial congenital abnormality that affects the cerebellum and some of its components; particularly cerebellar vermis, fourth ventricle and is characterized by an enlarged posterior fossa. Although there is an extensive list of signs attributed to DWM, final diagnosis is solely dependent on imaging techniques as there are no signs that are characteristic of DWM. This article reports a case with DWM who was diagnosed by magnetic resonance imaging.
Subject(s)
Dandy-Walker Syndrome/diagnosis , Dandy-Walker Syndrome/epidemiology , Hypertelorism , Magnetic Resonance Imaging/methods , Female , Humans , Palate/abnormalities , Young AdultABSTRACT
Neurocutaneous melanosis (NCM) is a rare congenital disease that is characterized by the presence of large or multiple congenital melanocytic nevi and melanotic lesions of the central nervous system. We report here on the CT and MR imaging findings of an unusual case of NCM that was associated with intraventricular dermoid and Dandy-Walker malformation.