Intestinal volvulus in the pump twin of a twin reversed arterial perfusion (TRAP) sequence after laser therapy at 18 weeks: a case report.

BACKGROUND: Twin reversed arterial perfusion sequence is a rare and potentially lethal condition affecting approximately 1% of monochorionic twin pregnancies and 1 in 35,000 pregnancies overall. An apparently normal (pump) twin perfuses its severely malformed cotwin with deoxygenated blood via retrograde flow in direct arterioarterial anastomoses between the umbilical arteries of each twin. Fetal intestinal volvulus is a rare condition usually manifesting after birth. We report a unique case of twin reversed arterial perfusion sequence in association with intestinal volvulus in the surviving pump twin. CASE PRESENTATION: A 32-year-old Hispanic primigravida was referred to our clinic after a fetoscopy procedure of laser photocoagulation of anastomoses at 18 weeks of gestation. Follow up scans in the ex-pump twin revealed dilated bowel loops and a typical "whirlpool sign" at 26 weeks of gestation, and intrauterine intestinal volvulus was suspected. At 29 weeks of gestation, preterm premature rupture of membranes occurred, and an emergency cesarean section was performed. The newborn was diagnosed in the early neonatal period with intestinal perforation. The diagnosis was postnatally confirmed by surgery and histopathology. CONCLUSIONS: The type of fetal intervention and late gestational age of the procedure increase the risk of complications. This case alerts health providers to be vigilant in the follow-up of patients with complicated monochorionic pregnancies.

Poliposis adenomatosos familiar con transformación maligna en niños: presentación de casos

La poliposis adenomatosa familiar (PAF) y su variante, el Síndrome de Turcot, son heredados de forma autosómica dominante por mutaciones en el gen APC (adenomatous polyposis coli). Ocurre en aproximadamente 1/10000 a 1/30000 nacidos vivos y representa menos del 1% del riesgo de cáncer colorrectal en Estados Unidos. El cáncer colorrectal debe ser considerado una consecuencia inevitable de la evolución natural de la PAF. Sin embargo, en casos familiares se puede promover una detección temprana. Presentamos tres casos de pacientes con PAF con transformación maligna dos de ellos hermanos, uno de los cuales desarrolló síndrome de Turcot y el tercer caso diagnosticado por antecedente en la madre de PAF además de ser estudiada por anemia en clínica de hematología.

Familial adenomatous polyposis (FAP) and its variant Turcot's syndrome are autosomal dominant diseases caused by mutations in the adenomatous polyposis coli (APC) gene. FAP occurs in approximately 1/10,000 to 1/30,000 live births, and accounts for less than 1 percent of the total colon cancer risk in the United States. Colorectal cancer should be considered an inevitable consequence of the natural history of FAP, but an early detection is possible in familial cases. We present three cases of FAP patients with malignant transformation including two brothers, one of whom developed Turcot syndrome and the third case diagnosed in screening for a history of PAF and mother with a history of anemia studied in hematology clinic.

Estudio comparativo de la morbi-mortalidad neonatal asociada los pretérminos tardíos y los recién nacidos a término en el Hospital del Niño

Metodología: Estudio retrospectivo, de casos y controles para comparar la morbilidad y mortalidad de los neonatos pretérminos tardíos (34 0/7 semanas a 36 6/7 semanas) en relación con los neonatos de término que fueron atendidos en el Hospital del Niño de Panamá, en el segundo semestre de 2011. Se tomaron todos los pretérminos tardíos en el período de estudio y se comparan con controles en relación 1:1. Los datos fueron obtenidos en la sección de Registros Estadísticos de Salud (REGES) del Hospital del Niño. Resultados: Hubo un total de 9118 nacimientos en este período, de los cuales 605 nacimientos fueron de pretérminos tardíos. Se excluyen 148 expedientes y se analizan 457 expedientes de cada grupo. Comparados con los neonatos de término, los pretérminos tardíos tienen mayor morbilidad (44.58% vs 8.10%): respiratoria (20.57% vs 1.75%), infecciosa (17.72% vs 2.63%), hipotermia (7.88% vs 0.44%), problemas de alimentación (6.78% vs 0.22%) y la Hiperbilirrubinemia que amerita fototerapia (22.92% vs 4.6%). Se encontró una mayor estancia intrahospitalaria (11.2 días vs 7.3 días) y costos intrahospitalarios (10 veces mayor). No se encontró asociación estadísticamente significativa en cuanto a hipoglicemia y mortalidad. Conclusión: los pretérminos tardíos tienen un riesgo importante de 11 veces más de desarrollar morbilidad y una mayor estancia intrahospitalaria generando más costos hospitalarios.

Methods: Case-control, retrospective study to compare the morbidity and mortality between late preterm infants (34 0/7 to 36 6/7 weeks of gestation) and full term infants that was attended in the Hospital del Niño of Panamá, in the period of second semester of 2011. All late preterm infants will be included and compared with 1:1 relationship to control term infants group. Data was obtained at Health Statistics Register (HSR) of Hospital del Niño. Results: During this study, born 9118 infants. 605 neonates born late preterm infants. Data of 148 late preterm infants were excluded and 457 infants were analyzed of each group. Compared with the full term infants, late preterm infants have a higher morbidity (44.58% vs 8.10%): respiratory distress (20.57% vs 1.75%), infectious problems (17.72% vs 2.63%), hypothermia (7.88% vs 0.44%), feeding problems (6.78% vs 0.22%) and jaundice requiring phototherapy (22.92% vs 4.6%). They have a higher hospitalization stay (mean, 11.2 days vs 7.3 days) and costs (10 times higher). Hypoglycemia and mortality was not statistically significant between the both groups. Conclusion: the late preterm has a significant risk of developing 11 times greater morbidity and hospital stay generating more hospital costs.