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      Prenatal Diagnosis of Fetal Encephalomalacia after Maternal Diabetic Ketoacidosis

      case-report

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          Abstract

          Introduction Encephalomalacia in a developing fetus is a rare and devastating neurological finding on radiologic imaging. Maternal diabetic ketoacidosis (DKA) can lead to metabolic and vascular derangements which can cause fetal encephalomalacia.

          Case We report the case of a 27-year-old pregnant woman with White's Class C diabetes mellitus who presented in the 25th week of gestation with DKA. Four weeks after her discharge, marked fetal cerebral ventriculomegaly was noted on ultrasound. A subsequent fetal magnetic resonance imaging (MRI) demonstrated extensive, symmetric cystic encephalomalacia, primarily involving both cerebral hemispheres. The pregnancy was continued with close fetal and maternal surveillance. The patient underwent a repeat cesarean delivery in her 37th week. The infant had a 1 month neonatal intensive care unit stay with care rendered by a multiple disciplinary team of pediatric subspecialists. The postnatal course was complicated by global hypotonia, poor feeding, delayed development and ultimately required anticonvulsants for recurrent seizures. He died at the age of 9 months from aspiration during a seizure.

          Discussion Although the maternal mortality from DKA has declined, DKA still confers significant neurological fetal morbidity to its survivors.

          Most cited references6

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          Neurobiology of periventricular leukomalacia in the premature infant.

          J Volpe (2001)
          Brain injury in the premature infant is a problem of enormous importance. Periventricular leukomalacia (PVL) is the major neuropathologic form of this brain injury and underlies most of the neurologic morbidity encountered in survivors of premature birth. Prevention of PVL now seems ultimately achievable because of recent neurobiologic insights into pathogenesis. The pathogenesis of this lesion relates to three major interacting factors. The first two of these, an incomplete state of development of the vascular supply to the cerebral white matter, and a maturation-dependent impairment in regulation of cerebral blood flow underlie a propensity for ischemic injury to cerebral white matter. The third major pathogenetic factor is the maturation-dependent vulnerability of the oligodendroglial (OL) precursor cell that represents the major cellular target in PVL. Recent neurobiologic studies show that these cells are exquisitely vulnerable to attack by free radicals, known to be generated in abundance with ischemia-reperfusion. This vulnerability of OLs is maturation-dependent, with the OL precursor cell highly vulnerable and the mature OL resistant, and appears to relate to a developmental window characterized by a combination of deficient antioxidant defenses and active acquisition of iron during OL differentiation. The result is generation of deadly reactive oxygen species and apoptotic OL death. Important contributory factors in pathogenesis interact with this central theme of vulnerability to free radical attack. Thus, the increased likelihood of PVL in the presence of intraventricular hemorrhage could relate to increases in local iron concentrations derived from the hemorrhage. The important contributory role of maternal/fetal infection or inflammation and cytokines in the pathogenesis of PVL could be related to effects on the cerebral vasculature and cerebral hemodynamics, to generation of reactive oxygen species, or to direct toxic effects on vulnerable OL precursors. A key role for elevations in extracellular glutamate, caused by ischemia-reperfusion, is suggested by demonstrations that glutamate causes toxicity to OL precursors by both nonreceptor- and receptor-mediated mechanisms. The former involves an exacerbation of the impairment in antioxidant defenses, and the latter, an alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid/kainate receptor-mediated cell death. Most importantly, these new insights into the pathogenesis of PVL suggest potential preventive interventions. These include avoidance of cerebral ischemia by detection of infants with impaired cerebrovascular autoregulation, e.g. through the use of in vivo near-infrared spectroscopy, the use of free radical scavengers to prevent toxicity by reactive oxygen species, the administration of alpha-amino-3-hydroxy-5-methyl-4-isoxazolepropionic acid/kainate receptor antagonists to prevent glutamate-mediated injury, or the use of maternal antibiotics or anticytokine agents to prevent toxicity from maternal/fetal infection or inflammation and cytokines.
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            Mechanisms of perinatal brain injury.

            This article is focused on the mechanisms underlying primarily ischaemic/reperfusion brain injury in both the term and premature infant. Although the mechanisms involved include similar initiating events, principally ischaemia-reperfusion, and similar final common pathways to cell death, particularly free radical-mediated events, there are certain unique maturational factors influencing the type and pattern of cellular injury. We will therefore initially describe the physiological and cellular/molecular mechanisms of brain injury in the term infant, followed by the mechanisms in the premature infant. Copyright 2000 Harcourt Publishers Ltd.
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              Diabetes ketoacidosis in pregnancy.

              Diabetic ketoacidosis (DKA) is a serious medical and obstetrical emergency usually occurring in patients with type 1 (insulin-dependent) diabetes mellitus. Although modern management of the patient with diabetes should prevent the occurrence of DKA during pregnancy, this complication still occurs and can result in significant morbidity and mortality for mother and/or fetus. Metabolic changes occurring during pregnancy can predispose a pregnant diabetic to DKA. The diagnosis of DKA can be more challenging during pregnancy as it does not always manifest with the classic presenting symptoms or laboratory findings. In fact, although uncommon, during pregnancy, DKA may develop even in the setting of relative normoglycemia. Prompt diagnosis and management is essential in order to optimize maternal and fetal outcomes. This article will provide the reader with information regarding the pathophysiology underlying DKA complicating pregnancy and will provide practical management guidelines for the diagnosis and management of this condition.
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                Author and article information

                Journal
                AJP Rep
                AJP Rep
                10.1055/s-00000169
                AJP Reports
                Thieme Medical Publishers (333 Seventh Avenue, New York, NY 10001, USA. )
                2157-6998
                2157-7005
                18 November 2014
                November 2014
                : 4
                : 2
                : e97-e100
                Affiliations
                [1 ]Department of Obstetrics and Gynecology, Naval Medical Center, Portsmouth, Virginia
                [2 ]Department of Obstetrics and Gynecology, Langley Air Force Base, Virginia
                [3 ]Department of Radiology, Naval Medical Center. Portsmouth, Virginia
                Author notes
                Address for correspondence Amy Lee, MD 925 Drivers Ln Newport News, VA 23602 amymilee@ 123456gmail.com
                Article
                140031
                10.1055/s-0034-1395990
                4239142
                260bd439-45e5-42b0-9da1-bc7142dfae3e
                © Thieme Medical Publishers
                History
                : 28 June 2014
                : 18 September 2014
                Categories
                Article

                diabetic pregnancy,diabetic ketoacidosis,encephalomalacia

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