Visual Diagnoses: Swollen, Discolored Umbilical Cord Stump

A female term newborn presents at birth with a section of the umbilical cord that appears to be edematous and filled with blood.Born to a 28-year-old gravida 1, para 1 woman at an estimated 39 weeks 4 days gestational agePrenatal laboratory findings: Group B Streptococcus positive test result, rapid plasma reagin reactive with a titer 1:1, fluorescent treponemal antibody-absorption negative result, human immunodeficiency nonreactive, rubella immune, hepatitis B nonreactive, hepatitis C nonreactive, Toxoplasma immunoglobulin G and M negative resultsPregnancy complicated by gestational hypertensionBorn via vaginal delivery, induction of labor for maternal hypertension (per obstetrical notes)Delayed cord clamping for 2 minutesNo maternal fever or signs of chorioamnionitis; 4 doses of ampicillin given to birth parent because of positive group B Streptococcus statusBirth weight of 3200 g (33rd percentile), length of 45.7 cm (3rd percentile), and head circumference of 33 cm (22nd percentile)Apgar scores of 8 and 9 were obtained at 1 and 5 minutes, respectively.Routine resuscitation was provided.The umbilical cord adjacent to the infant was found to be very edematous and filled with blood. The cord clamp was placed farther away than typical to avoid section with blood (Figure 1).The family declined erythromycin and requested oral vitamin K administration; after discussion with the family about bleeding risk despite oral vitamin K administration, the family was amenable to intramuscular vitamin K.Heart rate of 128 beats/minRespiratory rate of 76 breaths/min (tachypnea resolved within an hour)Temperature of 36.7°C (98.1°F)General: vigorous, strong cry, good color, normal activitySkin: warm-well perfusedHead: normocephalic, well-perfused, no atypical facial featuresEyes: red reflex present bilaterallyNose: nares patentMouth: moist mucous membranes, no cleft, no ankyloglossiaCardiovascular: regular rate and rhythm, no murmurs, femoral pulses 2+ and symmetricAbdominal: normoactive bowel sounds, soft, nondistended, no masses; edematous umbilical stump filled with blood; long, clamped umbilical cord segment, no discharge, no surrounding erythema, and no palpable herniated structuresGenitourinary: normal external female; no sacral pit, dimple, or tuft of hairExtremities: normal appearanceHips: negative Ortolani and BarlowNeuro: normal tone and normal reflexes including Moro, suck, graspWhite blood cell count: 29.7 K/MM3 (29.7 × 109/L)Hemoglobin: 19.6 g/dL (196 g/L)Platelet count: 211 K/MM3 (211 × 109/L)Ultrasound of umbilicus and cord remnant: unremarkable ultrasound of the umbilical region and cord remnant, and no focal collection or evidence of patent urachusWell-appearing term infant with proximal section of umbilical cord that was edematous with a dark purple hue. OmphaloceleUmbilical arteriovenous malformationUmbilical cord cyst (true cyst, pseudocyst)Umbilical cord hematoma (UCH)Umbilical knotUrachal anomaliesUCHThe infant remained stable throughout her admission. At 2 days of age, the infant’s umbilical cord blood dried up and the cord was reclamped and trimmed per parent preference. Another 2 clamps were added to the umbilical cord more proximal to the umbilicus. Then, the cord was cut in between the proximal clamps with minimal bleeding (Figure 2). The patient’s umbilical cord fell off at home with no complications.The umbilical cord is essential for the health and survival of the embryo or fetus as it serves as a lifeline, providing blood supply and serving as a waste removal system.1 UCHs are considered rare with an incidence rate of 1 in 5500 to 11 000 births.2 A UCH carries an increased risk of perinatal morbidity/mortality with approximately 50% resulting in fetal distress and stillbirth.2 Abnormal fetal heart monitor tracings with in utero UCHs have also been described.The pathophysiology of UCHs remains unknown. One hypothesis is that a spontaneous UCH results from disruption of the umbilical vessel walls leading to the extravasation of blood into the Wharton’s jelly, with high intravascular pressure possibly contributing to this process.3 A UCH can occur on any part of the umbilical cord but most often occurs near the umbilicus.4 Possible risk factors of UCHs include cord traction during labor, morphological anomalies of the umbilical cord, cord collapse, true knots, deficiency in Warton’s jelly, umbilical cord cysts, aneurysms, fetal clotting disorders, amniotic infection, abdominal trauma in pregnancy, and post maturity (Figure 3).3,5,6 Iatrogenic UCHs can also form from in utero instrumentation procedures including amniocentesis, fetal blood transfusions, and fetal diagnostic procedures.7 In this case, there was no definitive risk factor identified that led to the patient’s UCH but it is possible that cord traction during labor may have occurred as the bright blood of the UCH suggests an acute process (Figure 1).A UCH can arise prenatally or postnatally, although it is more frequently thought to happen during labor.3 The prenatal diagnosis of a UCH is possible, especially for large hematomas, with Doppler ultrasound.3 When detected prenatally, greater clinical attention is needed, as fetal hypoxia and anemia may occur secondary to compression of the umbilical vessels by the hematoma, potentially leading to perinatal asphyxia and stillbirth.3 However, UCHs are typically found incidentally after birth by physical examination,2 perhaps indicating they may occur closer to delivery. UCHs also often follow a benign course.8After birth and clamping of the umbilical cord, it has been suggested that an umbilical cord with a UCH does not require any additional care compared with an otherwise healthy umbilical cord and can be left to fall off on its own.9 Some have proposed evaluating infants with UCH for bleeding disorders;2 however, there does not seem to be consensus about this approach. On a case-to-case basis, it seems reasonable to screen certain infants with a UCH for a bleeding disorder, such as male infants who will undergo a circumcision, infants with other signs of bleeding, or in infants with a family history of a heritable bleeding disorder. Additionally, although an ultrasound is not required for diagnosis, it can be a helpful tool for excluding other diagnoses such as a patent urachus.SummaryA UCH is reportedly rare (1 in 5500–11 000 deliveries)2 and likely due to disruption of the umbilical vessels leading to the extravasation of blood into Wharton’s jelly.3Risk factors for a UCH include but are not limited to trauma,3 cord traction, amniotic infection, and in utero instrumentation procedures like amniocentesis.It is thought that at least 50% of cases of UCH result in fetal distress or stillbirth2 likely from fetal hypoxia and anemia that can result from compression of the umbilical vessels.3 Therefore, if detected prenatally, greater clinical attention is indicated.UCH is most often diagnosed incidentally on physical examination.2 If any doubts on diagnoses or concerns regarding contents within the umbilical cord, consider an ultrasound of the umbilical cord and abdomen.No special umbilical cord care is required for an infant with a UCH, but for a UCH involving a long section, consider placement of the clamp on section with minimal to no signs of blood. Once the cord starts to dry and the blood has become old, consider cutting the cord to minimize length.American Board of Pediatrics Neonatal-Perinatal Content SpecificationRecognize abnormalities of the umbilical cord.Know proper assessment of fetal well-being.