Perinatal hemorrhage from ulceration of the umbilical cord: A potentially catastrophic association with duodenal and jejunal obstruction.

PURPOSE: The purpose of this study is to review published reports and contribute new cases of umbilical cord ulceration (UCU) with perinatal hemorrhage into the amniotic cavity in the setting of duodenal or jejunal obstruction because knowledge of this sequence is poorly disseminated and could be lifesaving. METHODS: Published reports of UCU with hemorrhage associated with congenital duodenal or jejunal obstruction were reviewed. Chart review was conducted for the cases encountered at our institutions between January 2008 and March 2017. We noted perinatal complications, method of delivery, gestational age, birth weight, gender, number, location, and pathologic description of umbilical cord ulcers, and outcome. RESULTS: Thirty-one reports and 7 new cases were studied. Perinatal complications included: preterm labor or preterm premature rupture of membranes: 63%; fetal distress: 95%; mean gestational age: 33weeks; premature gestation: 95%; bloody amniotic fluid: 90%. Pathological analysis of UCUs revealed solitary, multifocal, helical and punched-out lesions. There were 12 neonatal deaths (32%), and 12 intrauterine deaths (32%). Survival rate was 37%. CONCLUSIONS: UCU with perinatal hemorrhage is associated with duodenal and jejunal obstruction. Knowing the typical clinical signs of this potential catastrophic complication could prompt lifesaving delivery. TYPE OF STUDY: Prognostic LEVEL OF EVIDENCE: IV.

Congenital Volkmann ischemic contracture: a case report.

Congenital Volkmann ischemic contracture is a rare condition in which a neonate presents with an ulcerated forearm, necrotic subcutaneous tissue, nerve palsy, and muscular necrosis. We report a case of a 1-day-old infant who presented with an ulceration of the left forearm noted at delivery combined with decreased motor function of the left distal forearm. Histologic examination of the ulceration revealed findings of subcutaneous fat necrosis of the newborn. To our knowledge, this is the first case for which the findings of subcutaneous fat necrosis have been seen histologically.

Umbilical cord ulcer associated with fetal jejunal atresia: report of 2 cases.

We report 2 cases of umbilical cord ulcer associated with fetal jejunal atresia. Both of them developed a severe intrauterine hemorrhage, followed by fetal heart rate decelerations, and underwent emergency cesarean section. Bloody amniotic fluid and umbilical cord ulcers were observed in both cases. Although both cases were successfully resuscitated, neurological impairment and renal failure developed in 1 case due to prolonged asphyxia. In a review of the literature, umbilical cord ulcer was associated only with congenital duodenal atresia or jejunal atresia, but not with ileal atresia. Although the prenatal diagnosis of duodenal or upper jejunal atresia has been established, the prenatal diagnosis of this complication has not been reported. In such cases, detailed examination of the umbilical cord by ultrasonography may be useful for the prenatal diagnosis of this disease.

Gastropatia hiperplasica glandular gigante (Enfermedad de Menetrier) / Menetrier´s diseases

La enfermedad de Ménétrier se caracteriza por un crecimiento glar dular con engrosamiento de pliegues en las paredes del estómago, constituyendo parte de un grupo de gastropatías hiperplásicas perdedoras de proteínas de etiología aún no precisada. Es una dolencia que ocurre usualmente en personas con edades entre 30 a 60 años, siendo muy rara en la edad pediátrica. Clinicamente se presenta con diarrea recurrente, vómitos, dolor abdominal y pérdida de peso. No existe un tratamiento específico, requiriendo solo un manejo sintomático de soporte. Los autores informan el caso de un niño de 7 años, con esta enfermedad, la cual se presentó con síntomas poco habituales (edemas, asciti derrame pleural); en los estudios de gabinete se confirmó PC trasonido, ascitis, derrame pleural y una hiperecogenecidad del estomago por la cual se procedió a realizar un tránsito gastrointestinal, cortando irregularidades en el llenado gástrico. El estadio endoscópico mostró un fundus gástrico de forma irregular, engrosando, con secreción espesa, viscosa y transparente en todo el estómago. El tratamiento intaurado consistió en la administración de albúmina, con la cual do obtener la desaparición de su sintomatología (edemas, ascitis ame pleural); posteriormente se continuó con la administració tomática de protectores gástricos y dieta hiperproteica. Fue dado d tres semanas despúes sin molestias significativas

Congenital palatal ulcers in newborn infants with cleft lip and palate: diagnosis, frequency, and significance.

Congenital decubital ulcers were found in 94% of newborn infants with unilateral cleft lip and palate in the course of a systematic study of a large cohort study (N = 52). The procedures for diagnosis, documentation, and follow-up are described. The ulceration area at birth varied over a wide range. The ulcerations were usually located in the posterior part of the vomer. Sonographic evidence supports the hypothesis that the ulcerations are caused mechanically by the motor activity of the tongue during the fetal and newborn period. The decubital ulcer disappeared in each case within 5 days following the implementation of a palatal plate.

Umbilical cord ulceration and intestinal atresia: a new association?

In three fetuses, congenital intestinal atresia was associated with linear ulcerations of the umbilical cord. In two cases, hemorrhage was seen from the cord ulcer. Both fetuses required emergency cesarean section for fetal distress and were born anemic. The third fetus was mildly hydropic, attributed to hemorrhage, and was stillborn. The mechanism of the association could not be determined. These cases suggest a risk of prenatal umbilical cord hemorrhage in infants with intestinal atresia.

Aplasia cutis congenita.

We report a family with aplasia cutis congenita, an extremely rare condition. Particular attention is paid to the clinical, etiologic, and genetic characteristics. The only possible treatment, as in the first case in this family, is excision and reconstruction by dermatologic surgery procedures.