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Hydrops fetalis and in utero intracranial hemorrhage
Volume 93 Number 6
3.
4.
Clinical notes
1023
hemorrhage-scalp of newborn infants, Am J Dis Child 108:413, 1964. Volpe JJ, Manica JP, Land VJ, and Loxe WE: Neonatal subdural hematoma association with severe hemophilia A, J PEDIATR 88:1023, 1976. Robinson RJ, and Rossiter MA: Massive subaponeurotic haemorrhage in babies of African origin, Arch Dis Child 43:684, 687, 1968.
Hydrops fetalis and in utero intracranial hemorrhage In 1943 Potter' identified a group of infants with hydrops fetalis which did not result from erythroblastosis. Greater interest in this group has evolved as the relative frequency of Rh isoimmunization declines. The recognized clinical conditions associated with nonimmunologic hydrops include congenital infections, malformations of the fetal lung, twinning, fetal tumors, homozygous a-chain thalassemia, malformations or dysfunction of the fetal cardiovascular system, thrombosis of major fetal or placental vessels, fetomaternal hemorrhage,' and red cell enzyme defects.' We report a previously unreported nonimmunologic cause of hydrops fetalis: massive in utero closed-space hemorrhage.
CASE REPORT A 3,330 gm female infant was born at 36 weeks' gestation to a 37-year-old mother who was first noted to have diabetes mellitus during this (fourth) pregnancy. and required up to 40 units of insulin daily for adequate control. She presented eight days prior to delivery in ketoacidosis and with severe vomiting which precipitated a Mallory-Weiss tear of her esophagus. The resultant hemorrhage and hypotension necessitated transfusion of three units of blood. Over the subsequent three days, gastrointestinal hemorrhage ceased, and oral feedings were initiated. During this time, fetal heart tones and intermittent fetal movements were observed, but no further assessment of fetal well-being was performed. One week after admission, on the day prior to delivery, an oxytocin challenge test demonstrated diminished variability of fetal heart rate in response to uterine contractions. The following day, the mother went into spontaneous labor and, after four hours, delivered vaginally without evidence of trauma. At delivery, the infant was flaccid, pale, and grossly edematous. She had a heart rate of 60/minute and no spontaneous respirations. Resuscitation included tracheal intubation, controlled ventilation, and administration of glucose via an umbilical venous catheter. The infant was assigned one- and five-minute Apgar scores of I and 3, respectively. The placenta appeared normal and weighed 560 gm. In the nursery, vital signs were normal except for the absence of spontaneous respirations. The weight was 3,300 gm (> 90%), the length 50 cm (75-90%), and the head circumference 37 cm (> 90%). There was no hepatosplenomegaly. The infant remained flaccid with no spontaneous movements and with fixed and dilated pupils. Serum electrolyte values on admission were: Na 131 mfiq/I, K 4.8 mEq/l, CI 96 mfiq/l, and CO, 12 mEq/l. The central hematocrit and hemoglo-
0022-34761781 J21023 +02$00.2010 © 1978 The C. V. Mosby Co.
Figure. Computerized tomography scan demonstrating hemorrhage in left temporal and parietal regions, and filling the lateral ventricles. bin values were 22% and 6.3 gm/dl, respectively, the reticulocyte count was 9.2% and the platelet count, 220,000Imm ''. Total serum protein concentration was 3.3 gm/dl, The maternal and infant blood type and group were both B positive; results of direct and indirect Coombs tests were negative. There was no serologic evidence of congenital infection and bacterial cultures were negative. The CSF evaluation demonstrated grossly bloody fluid with a protein content of 2,494 mg/dl. An immediate partial exchange transfusion with packed red blood cells was performed to raise the effective red cell mass. The infant was mechanically ventilated during this procedure, and subsequently computerized tomography (CT) demonstrated a massive hemorrhage involving the left parietal and temporal regions and filling the lateral ventricles (Figure). On the third day of life, after full discussion with the parents, mechanical support was discontinued because of the extent of the hemorrhage and the lack of neurologic improvement, and the infant died.
DISCUSSION In the series reported by Macaffee et al,' the incidence of nonimmunologic hydrops was one in 3,538 deliveries, constituting 17.6% of all hydropic babies. Among the nonimmunologic causes, loss of blood from the fetal circulation has been implicated in two settings: twin-to-twin transfusion' and fetomaternal hemorrhage.' In both, infants develop, to varying degrees, the classic signs of hydrops: edema, anemia, and hypoproteinemia. It is reasonable that loss of blood into a closed space might also precipitate the chain of events leading to hydrops. This patient apparently had such a closed-space hemorrhage. The period of time necessary to develop hydrops fetalis following the loss of fetal blood is unknown. In twinning and fetomaternal hemorrhage, blood loss probably occurs over a prolonged period of time. The same may have been true in this infant, although a single hemorrhagic event may have occurred coincident with the maternal hemorrhagic crisis. On CT scan,
1024
Clinical notes
The Journal oj Pediatrics December 1978
attenuation values (radiodensities) of intracerebral hemorrhages in adults are similar to those of adjacent cortical brain tissue within four to nine days and are thus no longer distinguishable.' The high radiodensities observed on this infant's scan suggest that there was either a single recent event or continued hemorrhage which began with the mother's hypotensive crisis. We concluded that sublethal, massive closed-space hemorrhage should be added to the previously reported causes of nonimmunologic hydrops fetalis,
Carl Bose, M.D. Division of Neonatal Medicine Department of Pediatrics University oj North Carolina School of Medicine Chapel Hill, NC 27514 REFERENCES 1. Potter EL: Universal edema of the fetus unassociated with erythroblastosis, Am J Obstet GynecoI46:130, 1943. 2. Driscoll SO: Hydrops fetalis, N Engl J Med 275: 1432, 1966, 3. Mentzer WC, and Collier E: Hydrops fetalis associated with erythorcyte G-6-PD deficiency and maternal ingestion of fava beans and ascorbic acid, J PEDIATR 86:565, 1975. 4. Macalfe CAJ, Fortune DW, and Beischer NA: Nonimmunologic hydrops fetalis, J Obstet Gynaecol Br Common 77:226, 1970. 5. Bergstrom M, Ericson K, Levander B, et al: Variation with time of the attenuation values of intracranial hematomas, J Comp Assist Torno (Comp Torno) 1:57, 1977.
Neonatal inguinoscrotal lesion produced by plastic umbilical clamp Plastic umbilical clamps are useful devices used all over the world. We have had the opportunity to observe in two patients a lesion probably produced by such a clamp, characterized by both inguinal and homolateral hemiscrotal hematomas. CASE REPORTS
Patient 1. This white, male, term infant, was the product of an uncomplicated gestation and normal vertex delivery. The neonatal examination was normal. On the second postpartum day, a left hemiscrotal hematoma was observed by the mother. On examination a homolateral hematoma was also appreciated in the inguinal region. Both were perfectly delimited, and palpation of the affected areas showed underlying subcutaneous infiltration. There was no apparent testicular involvement but thickening of the spermatic cord was noted. The lesion did not seem painful to palpation. Testicular appearance was normal by transillumination. There were no coagulation defects. Mother and infant were of the same blood group (A, Rh negative), The peak level of total bilirubin, on the fourth day of life, was 10 mg/dJ. The infant usually lay in the prone position. The round head of the umbilical plastic clamp could be matched perfectly in size and shape with the inguinal lesion. The device had been
0022·3476178/121024+02$00.2010
e
1978 The C. V. Mosby Co.
Figure. Patient 1. Inguinal hematoma with three circular elements of the same size and shape as the round head of the plastic clamp. The "secondary" scrotal hematoma is also visible. eccentrically clamped on the umbilical cord (Figure). When the infant was seen at the age of one month, the lesions were not apparent. Patient 2. This 9-day-old white male infant came to our emergency room because of a right hemiscrotal hematoma, first noticed on the third day of life. He had an identical lesion in the inguinal area as Patient I, but obviously discolored because of the time elapsed. There was no history of problems during pregnancy. The infant had a total bilirubin level of 14.5 mg/dl. The clamp was located very distally on the umbilical cord; its round head overlaid the inguinal lesion. The right testicle was apparently unaffected, and the spermatic cord thickened. The baby usually lay on his right side or on his abdomen. On further evaluation at the age of one month, the lesions had disappeared. DISCUSSION The lesions in both infants were probably due to compression of the umbilical plastic clamp on the inguinal venous superficial vasculature (scrotal branches of the femoral or saphenous veins ?); their rupture could have produced the inguinal hematoma. The appearance of the scrotal hematoma would be difficult to explain as a vascular consequence of the venous inguinal lesion; more probably it represents drainage of the inguinal hematoma to the scrotal area. We think that two factors playa pathogenic role: the infants' position, lying over the clamp, and incorrect clamping technique, eccentric to the cord (Patient I) or too distal (Patient 2), making possible the vascular compression between the clamp and the pubic arch. The appearance of the scrotal hematomas was sufficiently alarming that the inguinal lesion could have been ignored, missing the possibility of understanding the cause-effect relationship. Initially such lesions could be confused with other problems such as direct scrotal trauma or testicular torsion, We do not think that the lesions reported here have any serious consequence, but it is obviously important not to confuse these benign lesions with testicular torsion to avoid possible surgical proced ures.
3.
4.
Clinical notes
1023
hemorrhage-scalp of newborn infants, Am J Dis Child 108:413, 1964. Volpe JJ, Manica JP, Land VJ, and Loxe WE: Neonatal subdural hematoma association with severe hemophilia A, J PEDIATR 88:1023, 1976. Robinson RJ, and Rossiter MA: Massive subaponeurotic haemorrhage in babies of African origin, Arch Dis Child 43:684, 687, 1968.
Hydrops fetalis and in utero intracranial hemorrhage In 1943 Potter' identified a group of infants with hydrops fetalis which did not result from erythroblastosis. Greater interest in this group has evolved as the relative frequency of Rh isoimmunization declines. The recognized clinical conditions associated with nonimmunologic hydrops include congenital infections, malformations of the fetal lung, twinning, fetal tumors, homozygous a-chain thalassemia, malformations or dysfunction of the fetal cardiovascular system, thrombosis of major fetal or placental vessels, fetomaternal hemorrhage,' and red cell enzyme defects.' We report a previously unreported nonimmunologic cause of hydrops fetalis: massive in utero closed-space hemorrhage.
CASE REPORT A 3,330 gm female infant was born at 36 weeks' gestation to a 37-year-old mother who was first noted to have diabetes mellitus during this (fourth) pregnancy. and required up to 40 units of insulin daily for adequate control. She presented eight days prior to delivery in ketoacidosis and with severe vomiting which precipitated a Mallory-Weiss tear of her esophagus. The resultant hemorrhage and hypotension necessitated transfusion of three units of blood. Over the subsequent three days, gastrointestinal hemorrhage ceased, and oral feedings were initiated. During this time, fetal heart tones and intermittent fetal movements were observed, but no further assessment of fetal well-being was performed. One week after admission, on the day prior to delivery, an oxytocin challenge test demonstrated diminished variability of fetal heart rate in response to uterine contractions. The following day, the mother went into spontaneous labor and, after four hours, delivered vaginally without evidence of trauma. At delivery, the infant was flaccid, pale, and grossly edematous. She had a heart rate of 60/minute and no spontaneous respirations. Resuscitation included tracheal intubation, controlled ventilation, and administration of glucose via an umbilical venous catheter. The infant was assigned one- and five-minute Apgar scores of I and 3, respectively. The placenta appeared normal and weighed 560 gm. In the nursery, vital signs were normal except for the absence of spontaneous respirations. The weight was 3,300 gm (> 90%), the length 50 cm (75-90%), and the head circumference 37 cm (> 90%). There was no hepatosplenomegaly. The infant remained flaccid with no spontaneous movements and with fixed and dilated pupils. Serum electrolyte values on admission were: Na 131 mfiq/I, K 4.8 mEq/l, CI 96 mfiq/l, and CO, 12 mEq/l. The central hematocrit and hemoglo-
0022-34761781 J21023 +02$00.2010 © 1978 The C. V. Mosby Co.
Figure. Computerized tomography scan demonstrating hemorrhage in left temporal and parietal regions, and filling the lateral ventricles. bin values were 22% and 6.3 gm/dl, respectively, the reticulocyte count was 9.2% and the platelet count, 220,000Imm ''. Total serum protein concentration was 3.3 gm/dl, The maternal and infant blood type and group were both B positive; results of direct and indirect Coombs tests were negative. There was no serologic evidence of congenital infection and bacterial cultures were negative. The CSF evaluation demonstrated grossly bloody fluid with a protein content of 2,494 mg/dl. An immediate partial exchange transfusion with packed red blood cells was performed to raise the effective red cell mass. The infant was mechanically ventilated during this procedure, and subsequently computerized tomography (CT) demonstrated a massive hemorrhage involving the left parietal and temporal regions and filling the lateral ventricles (Figure). On the third day of life, after full discussion with the parents, mechanical support was discontinued because of the extent of the hemorrhage and the lack of neurologic improvement, and the infant died.
DISCUSSION In the series reported by Macaffee et al,' the incidence of nonimmunologic hydrops was one in 3,538 deliveries, constituting 17.6% of all hydropic babies. Among the nonimmunologic causes, loss of blood from the fetal circulation has been implicated in two settings: twin-to-twin transfusion' and fetomaternal hemorrhage.' In both, infants develop, to varying degrees, the classic signs of hydrops: edema, anemia, and hypoproteinemia. It is reasonable that loss of blood into a closed space might also precipitate the chain of events leading to hydrops. This patient apparently had such a closed-space hemorrhage. The period of time necessary to develop hydrops fetalis following the loss of fetal blood is unknown. In twinning and fetomaternal hemorrhage, blood loss probably occurs over a prolonged period of time. The same may have been true in this infant, although a single hemorrhagic event may have occurred coincident with the maternal hemorrhagic crisis. On CT scan,
1024
Clinical notes
The Journal oj Pediatrics December 1978
attenuation values (radiodensities) of intracerebral hemorrhages in adults are similar to those of adjacent cortical brain tissue within four to nine days and are thus no longer distinguishable.' The high radiodensities observed on this infant's scan suggest that there was either a single recent event or continued hemorrhage which began with the mother's hypotensive crisis. We concluded that sublethal, massive closed-space hemorrhage should be added to the previously reported causes of nonimmunologic hydrops fetalis,
Carl Bose, M.D. Division of Neonatal Medicine Department of Pediatrics University oj North Carolina School of Medicine Chapel Hill, NC 27514 REFERENCES 1. Potter EL: Universal edema of the fetus unassociated with erythroblastosis, Am J Obstet GynecoI46:130, 1943. 2. Driscoll SO: Hydrops fetalis, N Engl J Med 275: 1432, 1966, 3. Mentzer WC, and Collier E: Hydrops fetalis associated with erythorcyte G-6-PD deficiency and maternal ingestion of fava beans and ascorbic acid, J PEDIATR 86:565, 1975. 4. Macalfe CAJ, Fortune DW, and Beischer NA: Nonimmunologic hydrops fetalis, J Obstet Gynaecol Br Common 77:226, 1970. 5. Bergstrom M, Ericson K, Levander B, et al: Variation with time of the attenuation values of intracranial hematomas, J Comp Assist Torno (Comp Torno) 1:57, 1977.
Neonatal inguinoscrotal lesion produced by plastic umbilical clamp Plastic umbilical clamps are useful devices used all over the world. We have had the opportunity to observe in two patients a lesion probably produced by such a clamp, characterized by both inguinal and homolateral hemiscrotal hematomas. CASE REPORTS
Patient 1. This white, male, term infant, was the product of an uncomplicated gestation and normal vertex delivery. The neonatal examination was normal. On the second postpartum day, a left hemiscrotal hematoma was observed by the mother. On examination a homolateral hematoma was also appreciated in the inguinal region. Both were perfectly delimited, and palpation of the affected areas showed underlying subcutaneous infiltration. There was no apparent testicular involvement but thickening of the spermatic cord was noted. The lesion did not seem painful to palpation. Testicular appearance was normal by transillumination. There were no coagulation defects. Mother and infant were of the same blood group (A, Rh negative), The peak level of total bilirubin, on the fourth day of life, was 10 mg/dJ. The infant usually lay in the prone position. The round head of the umbilical plastic clamp could be matched perfectly in size and shape with the inguinal lesion. The device had been
0022·3476178/121024+02$00.2010
e
1978 The C. V. Mosby Co.
Figure. Patient 1. Inguinal hematoma with three circular elements of the same size and shape as the round head of the plastic clamp. The "secondary" scrotal hematoma is also visible. eccentrically clamped on the umbilical cord (Figure). When the infant was seen at the age of one month, the lesions were not apparent. Patient 2. This 9-day-old white male infant came to our emergency room because of a right hemiscrotal hematoma, first noticed on the third day of life. He had an identical lesion in the inguinal area as Patient I, but obviously discolored because of the time elapsed. There was no history of problems during pregnancy. The infant had a total bilirubin level of 14.5 mg/dl. The clamp was located very distally on the umbilical cord; its round head overlaid the inguinal lesion. The right testicle was apparently unaffected, and the spermatic cord thickened. The baby usually lay on his right side or on his abdomen. On further evaluation at the age of one month, the lesions had disappeared. DISCUSSION The lesions in both infants were probably due to compression of the umbilical plastic clamp on the inguinal venous superficial vasculature (scrotal branches of the femoral or saphenous veins ?); their rupture could have produced the inguinal hematoma. The appearance of the scrotal hematoma would be difficult to explain as a vascular consequence of the venous inguinal lesion; more probably it represents drainage of the inguinal hematoma to the scrotal area. We think that two factors playa pathogenic role: the infants' position, lying over the clamp, and incorrect clamping technique, eccentric to the cord (Patient I) or too distal (Patient 2), making possible the vascular compression between the clamp and the pubic arch. The appearance of the scrotal hematomas was sufficiently alarming that the inguinal lesion could have been ignored, missing the possibility of understanding the cause-effect relationship. Initially such lesions could be confused with other problems such as direct scrotal trauma or testicular torsion, We do not think that the lesions reported here have any serious consequence, but it is obviously important not to confuse these benign lesions with testicular torsion to avoid possible surgical proced ures.