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Multiple thromboses in an infant of a diabetic mother
982
June 1977 TheJournalofPEDIATRICS
Multiple thromboses in an infant of a diabetic mother T h o m a s F. W a r d , M . D , , F . R . C , P . ( C ) , S a s k a t o o n , Sask., C a n a d a
THE INCREASED INCIDENCE of t h r o m b o s i s or t h r o m b o e m b o l i c p h e n o m e n a in the infants o f diabetic m o t h e r s c o m p a r e d to the infants o f n o n d i a b e t i c m o t h e r s is well recognized? R e n a l vein t h r o m b o s i s is c o m m o n 2. 3; o t h e r t h r o m b o t i c lesions such as g a n g r e n e o f the a r m h a v e b e e n reported, 4 as well as a previous case o f peripheral g a n g r e n e associated with renal vein t h r o m b o s i s ? T h e following case d o c u m e n t s a n i n f a n t w h o presented in the i m m e d i a t e p o s t p a r t u m period with a renal vein thrombosis a n d a g a n g r e n o u s lesion of his right forearm; subsequently he also h a d evidence of a cerebral t h r o m botic episode.
ting studies and platelet count, were within normal limits, as were the urea and creatinine values. The initial diagnosis was a right renal vein thrombosis and a thrombosis of the venous circulation of the right forearm. On the second day of life he had generalized seizures which were controlled with phenobarbital. A lumbar puncture was performed, and blood glucose and calcium determinations were obtained; these were all within normal limits. The phenobarbital was discontinued four days after commencement, with no return of the seizures. The mass in the right flank gradually decreased in firmness. A repeat urinalysis at one week of age revealed a protein content of 100 mg/dl with no RBCs or WBCs. One week later the urinalysis was completely normal. An IVP was repeated at one month and showed bilateral renal function.
CASE REPORT A 39-year-old para 4, gravida 9, diabetic woman was admitted at 35 weeks' gestation for control of diabetes. At 37 weeks' gestation, when investigation revealed a mature fetus, an elective induction was commenced; cesarean section was subsequently performed. A male infant weighing 3,720 gm was delivered; Apgar scores were 6 at one minute and 9 at five minutes. Examintion at the time of delivery revealed a firm liver palpable 2 c m b e l o w the right costal margin, a firm mass measuring 4 x 5 cm in the right flank, as well as a lesion on the right forearm. Most of the right forearm was covered by flaccid bullae with dark, well-demarcated margins (Fig. 1,A ). The tissue beneath the buUae appeared to be bluish gray in color. The radial, brachial, and axiUary pulses were not palpable, nor were they heard by the Doppler method. The hand distal to the lesion was cyanotic and swollen. Urinalysis revealed 1 to 2 RBCs and 1 to 2 WBCs per highpower field; an IVP, a nonfunctioning right kidney. The BP was normal. Over the next 24 hours the color gradually reappeared in the hand, and subsequently axillary, brachial, and radial pulses were heard. Results of laboratory investigations, including clot-
From the Department of Pediatrics, University Hospital. Reprint address: Department of Pediatrics, University Hospital, Saskatoon, Saskatchewan S7N OW8, Canada.
Vol. 90, No. 6, pp 982-984
Abbreviations used RBC: red blood cells WBC: white blood cells IVP: intravenous pyelogram EEG: electroencephalogram The bullae on the right forearm drained, and subsequently a black, leathery eschar began to form, encircling the right forearm. This decreased in size and was subsequently split at four weeks of age and then removed surgically at six weeks of age (Fig. I,B). At this time the right hand had become extremely edematous but had a normal color. No movements could be elicited in the hand, and there was no evidence of sensation. As the edema became more marked and the hand became cyanotic, an amputation of the forearm was performed at two months of age. During the first month of life a third problem presented. As the infant began to increase in weight and length, it was noted that his head circumference was not increasing at the expected rate, By one month of age the head circumference had increased only 1 cm from 33 to 34 cm and had dropped from the twenty-fifth to the third percentile. A neurologic examination, brain scan, and an EEG were within normal limits. The infant was discharged at the age of ten weeks. The right forearm was healing nicely, but the head circumference had increased only 2 cm from birth. He was subsequently seen at 39~ months of age at which time neurologic and developmental
Volume 90 Number 6
Multiple thromboses
983
Fig. 1. A, Photograph at two days of age showing lines of demarcation below elbow and at the wrist. Bullae has ruptured and eschar is beginning to form. B, Photograph at 40 days of age tbllowing surgical debridement of the eschar. examinations were approximately two to four weeks delayed. Head circumference was now 37 cm and was below the third percentile. He returned to hospital at the age of seven months for investigation of momentary spasms during the past two months. These spasms were described as involving extension of the arms and flexion of the head on the trunk. There was no movement of the lower limbs, cyanosis, or apnea. These spasms were often repeated two to three times in quick succession but lasted for only a few seconds. Neurologic examination and developmental testing showed the patient's development to be at the 3~/z-month level. On examination no focal signs were found but the anterior fontanelle, though open, measured only 0.5 • 0.5 era. The head circumference was 39 cm (average for age 44 cm), well below the third percentile, while the weight and length remained on the twenty-fifth percentile. A skull roentgenogram revealed cranial asymmetry, with a lacunar pattern over the parietal region, particularly on the left side. An EEG revealed decreased voltages over the left hemisphere, and a brain scan showed an increased distance between the cortex and calvarium on the left side. It was felt that these findings were secondary to cerebral infarction and atrophy. Although no further spasms were noted in hospital, the child was placed on phenobarbital and discharged home. Histologic studies of the amputated forearm revealed wellorganized arterial and venous thromboses at the margins on the
gangrenous area. In addition, axonal degeneration was noted in the nerve supply to the hand. COMMENT The association o f renal vein thrombosis in the n e w b o r n with m a t e r n a ! diabetes has b e e n reported by several a u t h o r s ? :~ N o n o p e r a t i v e t r e a t m e n t in the n e w b o r n infant has b e e n successful previously? Anticoagulative t h e r a p y was not e m p l o y e d because the platelet count was 131,000 m m :~ a n d the partial t h r o m b o p l a s t i n time was twice that o f the control. T h e g a n g r e n o u s lesion on the right f o r e a r m was t h o u g h t to be d u e to v e n o u s thrombosis r a t h e r t h a n to any arterial e m b o l u s from the renal vein t h r o m b u s . Evidence of m i c r o c e p h a l y was n o t e d early in the child's course, but the initial investigations revealed n o a b n o r malities. A t the age of seven months, however, there were signs o f cerebral atrophy, most m a r k e d in the left hemisphere. It is t h o u g h t that cerebral t h r o m b o s i s is the p r o b a b l e e x p l a n a t i o n for the seizures early in the n e o n a t a l period a n d the spasms that b e g a n at the age o f five months.
984
Ward
REFERENCES 1. Oppenheimer E, and Esterly J: Thrombosis in the newborn: Comparison between infants of diabetic and non-diabetic mothers, J PEDIATR 67:549, 1965. 2. Avery M, Oppenheimer E, and Gordon H: Renal vein thrombosis in newborn infants of diabetic mothers, N Engl J Med 256:1134, 1957. 3. Takeuchi A, and Berirschke K: Renal venous thrombosis of the newborn and its relation to maternal diabetes, Biol Neonate 3:237, 1961.
The Journal of Pediatrics June 1977
4.
Hensinger RW: Gangrene of the newborn: A case report, J Bone Joint Surg 57:121, 1975. 5. Valderrama E, Gribetz I, and Strauss L: Peripheral gangrene in a newborn infant associated with renal and adrenal vein thrombosis, J P~Ol^TR 80:101, 1972. 6. Blemen A, Susmano D, Burden J, and Kaplan G: Nonoperative treatment of unilateral renal vein thrombosis in the newborn, JAMA 211:1165, 1970.
June 1977 TheJournalofPEDIATRICS
Multiple thromboses in an infant of a diabetic mother T h o m a s F. W a r d , M . D , , F . R . C , P . ( C ) , S a s k a t o o n , Sask., C a n a d a
THE INCREASED INCIDENCE of t h r o m b o s i s or t h r o m b o e m b o l i c p h e n o m e n a in the infants o f diabetic m o t h e r s c o m p a r e d to the infants o f n o n d i a b e t i c m o t h e r s is well recognized? R e n a l vein t h r o m b o s i s is c o m m o n 2. 3; o t h e r t h r o m b o t i c lesions such as g a n g r e n e o f the a r m h a v e b e e n reported, 4 as well as a previous case o f peripheral g a n g r e n e associated with renal vein t h r o m b o s i s ? T h e following case d o c u m e n t s a n i n f a n t w h o presented in the i m m e d i a t e p o s t p a r t u m period with a renal vein thrombosis a n d a g a n g r e n o u s lesion of his right forearm; subsequently he also h a d evidence of a cerebral t h r o m botic episode.
ting studies and platelet count, were within normal limits, as were the urea and creatinine values. The initial diagnosis was a right renal vein thrombosis and a thrombosis of the venous circulation of the right forearm. On the second day of life he had generalized seizures which were controlled with phenobarbital. A lumbar puncture was performed, and blood glucose and calcium determinations were obtained; these were all within normal limits. The phenobarbital was discontinued four days after commencement, with no return of the seizures. The mass in the right flank gradually decreased in firmness. A repeat urinalysis at one week of age revealed a protein content of 100 mg/dl with no RBCs or WBCs. One week later the urinalysis was completely normal. An IVP was repeated at one month and showed bilateral renal function.
CASE REPORT A 39-year-old para 4, gravida 9, diabetic woman was admitted at 35 weeks' gestation for control of diabetes. At 37 weeks' gestation, when investigation revealed a mature fetus, an elective induction was commenced; cesarean section was subsequently performed. A male infant weighing 3,720 gm was delivered; Apgar scores were 6 at one minute and 9 at five minutes. Examintion at the time of delivery revealed a firm liver palpable 2 c m b e l o w the right costal margin, a firm mass measuring 4 x 5 cm in the right flank, as well as a lesion on the right forearm. Most of the right forearm was covered by flaccid bullae with dark, well-demarcated margins (Fig. 1,A ). The tissue beneath the buUae appeared to be bluish gray in color. The radial, brachial, and axiUary pulses were not palpable, nor were they heard by the Doppler method. The hand distal to the lesion was cyanotic and swollen. Urinalysis revealed 1 to 2 RBCs and 1 to 2 WBCs per highpower field; an IVP, a nonfunctioning right kidney. The BP was normal. Over the next 24 hours the color gradually reappeared in the hand, and subsequently axillary, brachial, and radial pulses were heard. Results of laboratory investigations, including clot-
From the Department of Pediatrics, University Hospital. Reprint address: Department of Pediatrics, University Hospital, Saskatoon, Saskatchewan S7N OW8, Canada.
Vol. 90, No. 6, pp 982-984
Abbreviations used RBC: red blood cells WBC: white blood cells IVP: intravenous pyelogram EEG: electroencephalogram The bullae on the right forearm drained, and subsequently a black, leathery eschar began to form, encircling the right forearm. This decreased in size and was subsequently split at four weeks of age and then removed surgically at six weeks of age (Fig. I,B). At this time the right hand had become extremely edematous but had a normal color. No movements could be elicited in the hand, and there was no evidence of sensation. As the edema became more marked and the hand became cyanotic, an amputation of the forearm was performed at two months of age. During the first month of life a third problem presented. As the infant began to increase in weight and length, it was noted that his head circumference was not increasing at the expected rate, By one month of age the head circumference had increased only 1 cm from 33 to 34 cm and had dropped from the twenty-fifth to the third percentile. A neurologic examination, brain scan, and an EEG were within normal limits. The infant was discharged at the age of ten weeks. The right forearm was healing nicely, but the head circumference had increased only 2 cm from birth. He was subsequently seen at 39~ months of age at which time neurologic and developmental
Volume 90 Number 6
Multiple thromboses
983
Fig. 1. A, Photograph at two days of age showing lines of demarcation below elbow and at the wrist. Bullae has ruptured and eschar is beginning to form. B, Photograph at 40 days of age tbllowing surgical debridement of the eschar. examinations were approximately two to four weeks delayed. Head circumference was now 37 cm and was below the third percentile. He returned to hospital at the age of seven months for investigation of momentary spasms during the past two months. These spasms were described as involving extension of the arms and flexion of the head on the trunk. There was no movement of the lower limbs, cyanosis, or apnea. These spasms were often repeated two to three times in quick succession but lasted for only a few seconds. Neurologic examination and developmental testing showed the patient's development to be at the 3~/z-month level. On examination no focal signs were found but the anterior fontanelle, though open, measured only 0.5 • 0.5 era. The head circumference was 39 cm (average for age 44 cm), well below the third percentile, while the weight and length remained on the twenty-fifth percentile. A skull roentgenogram revealed cranial asymmetry, with a lacunar pattern over the parietal region, particularly on the left side. An EEG revealed decreased voltages over the left hemisphere, and a brain scan showed an increased distance between the cortex and calvarium on the left side. It was felt that these findings were secondary to cerebral infarction and atrophy. Although no further spasms were noted in hospital, the child was placed on phenobarbital and discharged home. Histologic studies of the amputated forearm revealed wellorganized arterial and venous thromboses at the margins on the
gangrenous area. In addition, axonal degeneration was noted in the nerve supply to the hand. COMMENT The association o f renal vein thrombosis in the n e w b o r n with m a t e r n a ! diabetes has b e e n reported by several a u t h o r s ? :~ N o n o p e r a t i v e t r e a t m e n t in the n e w b o r n infant has b e e n successful previously? Anticoagulative t h e r a p y was not e m p l o y e d because the platelet count was 131,000 m m :~ a n d the partial t h r o m b o p l a s t i n time was twice that o f the control. T h e g a n g r e n o u s lesion on the right f o r e a r m was t h o u g h t to be d u e to v e n o u s thrombosis r a t h e r t h a n to any arterial e m b o l u s from the renal vein t h r o m b u s . Evidence of m i c r o c e p h a l y was n o t e d early in the child's course, but the initial investigations revealed n o a b n o r malities. A t the age of seven months, however, there were signs o f cerebral atrophy, most m a r k e d in the left hemisphere. It is t h o u g h t that cerebral t h r o m b o s i s is the p r o b a b l e e x p l a n a t i o n for the seizures early in the n e o n a t a l period a n d the spasms that b e g a n at the age o f five months.
984
Ward
REFERENCES 1. Oppenheimer E, and Esterly J: Thrombosis in the newborn: Comparison between infants of diabetic and non-diabetic mothers, J PEDIATR 67:549, 1965. 2. Avery M, Oppenheimer E, and Gordon H: Renal vein thrombosis in newborn infants of diabetic mothers, N Engl J Med 256:1134, 1957. 3. Takeuchi A, and Berirschke K: Renal venous thrombosis of the newborn and its relation to maternal diabetes, Biol Neonate 3:237, 1961.
The Journal of Pediatrics June 1977
4.
Hensinger RW: Gangrene of the newborn: A case report, J Bone Joint Surg 57:121, 1975. 5. Valderrama E, Gribetz I, and Strauss L: Peripheral gangrene in a newborn infant associated with renal and adrenal vein thrombosis, J P~Ol^TR 80:101, 1972. 6. Blemen A, Susmano D, Burden J, and Kaplan G: Nonoperative treatment of unilateral renal vein thrombosis in the newborn, JAMA 211:1165, 1970.