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INTRODUCTION: A cloaca occurs when genitourinary tract and bowel converge into a common channel. We report a case of partial caudal duplication, persistent cloaca and vestigial appendage in a monovular female twin infant. PRESENTATION OF CASE: This is a monochorinonic-diamniotic twin born at 36 weeks with apgars of 9/9. She had a duplicated labia with two clitorises, and a partially formed accessory foot with 2 toes protruding from the right gluteal region. There was anal atresia and a punctate urethral opening in the right genitalia through which she voided spontaneously. X-ray of the accessory foot had rudimentary metatarsals and phalanges. There was left hydroureteronephrosis and a hydrocolpos causing severe mass effect. On the first day of life, she had exploratory laparotomy with a diverting colostomy and mucus fistula and drainage of hydrocolpos. At 6 months of age, she had removal of the accessory foot with flap closure of the perineal defect and vesicostomy. At 15 months of age she had laparotomy for repair of cloaca, excision of presacral pelvic mass and the duplicated vulva. DISCUSSION: Theories of etiology include failure of regression of Kovalevsky's canal (a communication that connects the amniotic and yolk sac), an incomplete form of twinning through iatrogenic damage to the zona pellucida or a failed triplet formation from a single embryo. CONCLUSION: Caudal duplication with persistent cloaca and vestigial appendage is a rare and complex malformation. Having a unified surgical and medical team to preserve quality of life and to treat complications is of key importance.
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Periventricular leukomalacia (PVL), a brain injury affecting premature infants is commonly associated with cerebral palsy. PVL results from hypoxia-ischemia (HI) with or without infection and is characterized by white matter necrotic lesions, hypomyelination, microglial activation, astrogliosis, and neuronal death. It is important to study a PVL mouse model that mimics human PVL in symptomatology, anatomic and molecular basis. In our neonate mice model, bilateral carotid arteries were temporary ligated at P5 followed by hypoxic exposure (FiO2 of 8% for 20 min.). At P5 in mice, the white matter is more vulnerable to HI injury than the grey matter. In our PVL model, mice suffer from significant hind limb paresis, incoordination and feeding difficulties. Histologically they present with ventriculomegally, white matter loss, microglial activation and neuronal apoptosis. HI injury increases proinflammtory cytokines, activates NF-kB, activates microglia and causes nitrative stress. All these inflammatory mediators lead to oligodendroglial injury and white matter loss. Neurobehavioral analysis in the PVL mice model at P60 showed that the HI group had a significant decrease in hind limb strength, worsening rotarod testing and worsening performance in the open field test. This new PVL model has great advantages far beyond just mimicking human PVL in clinical features and histopathology. Long term survival, the development of cerebral palsy and the ability of using this model in transgenic animals will increase our understanding of the mechanistic pathways underlying PVL and defining specific targets for the development of suitable therapeutics.
