Neurnradiolngv
Neuroradiology16, 76-78 (1978)
© by Springer-Verlag1978
Radiography of Hydrocephalus after Total Parenteral Nutrition S. Puljic, L. J. Newman, L. Heitlinger, M. Y. Chaudhary, E. Hiesiger, and D. Kotsilimbas The Departments of Pediatrics, Radiology,and Neurosurgery,New York MedicalCollege,New York, New York, USA
Summary. An infant with chronic diarrhea developed hydrocephalus following treatment with total parenteral nutrition (TPN) via jugular vein catheterization. Total parenteral nutrition is used when nutritional needs cannot be met adequately by oral alimentation. Serial computerized tomograms showed progression of communicating hydrocephalus. Superior sagittal sinograms demonstrated bilateral internal jugular vein occlusion with extensive venous eoUateralization. Lumboperitoneal shunt effectively decreased raised CSF pressure. A judicious approach to alternative venous routes for hyperalimentation is suggested. Radiographic delineation of communicating hydrocephalus by computerized tomography and superior sagittal sinography is presented.
Communicating hydrocephalus following bilateral jugular vein thrombosis as a result of hyperalimentation has recently become a focus of attention. Stewart et al. [6] reported .four cases in which the development of communicating hydrocephalus was related to the prolongation of total parenteral nutrition via jugular vein catheterization. However, there was no radiological evidence of venous occlusion. We report on a patient with hydrocephalus following jugular vein thrombosis. Both jugular veins were catheterized for the purpose of central parenteral alimentation. The importance of radiological investigation for diagnostic and prognostic purposes is emphasized.
Case Report A 4-month-old male infant presented with chronic diarrhea. He had been fed Similiac 20 ® until 2 weeks of age when he developed severe watery diarrhea. He was admitted to a community hospital in a state of moderate dehydration. The child was treated with intravenous
therapy to correct metabolic abnormalities and restore normal hydration. With reintroduction of oral alimentation, diarrhea recurred. His weight at 3 months was 3.6 kg. A diagnosis of chronic intractable diarrhea of infancy was made and central parenteral alimentation was started. A Silastic catheter was threaded into the right atrium from the right jugular vein and a solution of free amino acids (Freeamine®) and glucose was given. After 12 days' insertion of the catheter, the patient developed swelling about the face and right shoulder. The catheter was removed and inserted into the left internal jugular vein. After 7 days' insertion, swelling of the left side of the neck occurred. The rate of administration was then decreased, and oral alimentation with Pregestimil® was started. Since diarrhea worsened with oral feeding, the Pregestimil® was discontinued. Twenty-six days after insertion, the catheter in the left internal jugular vein was removed. We first saw the child when he was transferred to the Pediatric Nutrition Service at New York Medical College for further treatment. At this time the child, now 4 months of age, was well hydrated. The head circumference was 42 cm (50th percentile), weight was 4 kg (below 3rd percentile). On physical examination surgical scars of the neck, swelling of the neck, and puffiness of the eyelids were found. Neurological development w a s normal for his age. Results of laboratory studies revealed no fluid or metabolic abnormalities. A mild anemia was present. On an oral dietary regimen and intravenous replacement of continuing diarrheal losses, improvement ensued over a 3-week period and the child was discharged. Follow-up examinations at 5 months revealed a head circumference of 43 cm (between 50th and 75th percentile) and weight of 5.8 kg (3rd percentile). At 8 months the head circumference was 48 cm (97th percentile). A C T scan (Fig. la) showed communicating hydrocephalus of moderate degree. A ventrieuloperitoneal shunt was attempted, but the catheter was not placed in the ventricle. By 14 months of age, the head circumference was 53 cm. The anterior fontanelle was 0028-3940178/0016[0076/$01.00
S. Puljic et al.: Radiography of Hydrocephalusafter Total Parenteral Nutrition
77
Discussion
Fig. la. Initial CT scan at 8 months demonstrates moderate ventricular dilatation, widening of subarachnoid spaces especiallyinto the interhemispheric area. b. CT scan at 22 months shows mild ventricular dilatation and normal sulei
Fig. 2a. Lateral view of superior sagittal sinogram shows obstruction of internal jugular system with vertebral collateralization,b. Aiateroposteriorviewof superior sagittal sinogram shows obstruction of both jugular veins with venous eollaterization
open and bulging. The sutures had widened. A lumbar puncture was performed. The opening pressure was 290 mmH20; the closing pressure was 270 mmH20. A repeat CT scan showed progression of the hydrocephalus and cerebral edema. A carotid arteriogram to demonstrate a venous phase was not attempted; access to the carotid and femoral vessels was impeded by prior surgical explorations for venous cutdowns. A superior saglttal sinogram through 19-gauge butterfly needle (Fig. 2a, b) revealed obstruction of both internal jugular veins with extensive venous collateralization in the neck communicating with the superior vena cava. A lumboperitoneal shunt was inserted. During the ensuing 6 months the child developed normally. The head circumference remained 53.5 cm. The anterior fontanelle was no longer bulging and the sutures appeared normal. CT scans were almost normal (Fig. lb).
Communicating hydrocephalus is often acquired and may result from the impairment of resorption of cerebrospinal fluid at the level of arachnoid villi. Cerebrospihal fluid is absorbed into the venous system and any disturbance of the reabsorption process results in hydrocephalus. The mechanism by which a superior vena cava-like syndrome develops was clarified by Bering and Salibi [1] in studies in dogs where the entire cephalic venous outflow system was ligated, resulting in hydrocephalus. They postulated that i~ydrocephalus is due to the fact that increased venous pressure offsets the osmotic pressure in veins which in turn makes absorption of CSF difficult. In addition, increased pulsation of choroid plexuses contributes to the ventricular dilatation. Others [5] have shown that the collapse of the sagittal sinus, secondary to ventricular enlargement, further compromises venous outflow and decreases CSF absorption by the arachnoid villi. Possible compensatory mechanisms to relieve the hydrocephalus include the development of venous coUaterals in the emissary-external jugular systems and the vertebral plexus. This is brought about by the increased CSF pressure as well as the transventricular absorption of CSF. However, shunts which relieve this CSF pressure may also remove the stimulus for collateral development. Without shunting, ventricular expansion may lead to decompensated hydrocephalus [2, 4]. Haar and Miller [3] studied the problem of venous obstruction in hydrocephalic patients with dural sinus hypertension. They found that some of their patients had pseudotumor cerebri when the venous system was only partly occluded. In these cases, the ventricles were normal in size and the venous pressure was normal. Others had communicating hydrocephalus owing to bilateral venous outflow occlusion. In these cases, enlargement of the ventricles and increased intracranial venous pressure occurred. Our patient had features of both these types of cases. Central catheters were inserted into both internal jugular veins at different times for TPN. This resulted in a superior vena cava-like syndrome withventricular enlargement, cerebral edema, enlarged subarachnoid space, and dilatation of the cisterns. Perhaps this could have been avoided had the catheter been removed as the first signs of venous occlusion appeared. Because attempts to insert a ventriculoperitoneal shunt failed, the clinical signs of progressive hydrocephalus, i.e., increasing head circumference, bulging anterior fontanelle, and open suture lines, were persistent. The CSF pressure was exceedingly high (opening pressure 290 mmH20, closing pressure 270 mmH20). Serial CT scans demonstrated the increasing cerebral swelling and ventricular enlargement. Superior sagittal sinogram is an optional procedure to demonstrate jugular vein obstruction and venous collateralization. Manual injection of 15 cc of Renografin 60% through 19-gauge butterfly
78
S. Puljic et al.: Radiography of Hydrocephalus after Total Parenteral Nutrition
needle is sufficient to give excellent opacification. The procedure is fast, safe, easy, and well-tolerated. After a lumboperitonea[ shunt was inserted, the CSF pressure returned to normal; the anterial fontanelle became flattened. The rate o f head growth decreased. In the ensuing months the child developed normally. This report illustrates a complication of parenteral alimentation in infants with catheterization of the Cephalic venous outflow tracts. A judicious approach to alternative venous routes should be explored. The combined use of the computerized tomograms and the superior sagittal sinogram helped to delineate the type of hydrocephalus and the site o f venous obstruction. These diagnostic techniques showed the necessity for successful shunting in this patient.
2. Epstein, Z., Hochwald,G. M., Wald, A., Ransohoff, J.: Avoidance of shunt dependency in hydrocephalus. Dev. Med. Child Neurol. 17, 71-103 (1975) 3. Haar, E. L., Miller, C. A.: Hydrocephalus resulting from superior vena cava thrombosis in an infant. J. Neurosurg. 42, 597 to 601 (1975) 4. Hochwald, G. M., Epstein, F., Malhan, C., Ransohoff, J.: The relationship of compensated to decompensated hydrocephalus in the cat. J. Neurosurg. 39,694-697 (1973) 5. Shulman, K., Yarnell, R., Ransohoff, J.: Dural sinus pressure in normal and hydrocephalic dogs. Arch. Neurol. 81,575-580 (1964) 6. Stewart, D., Johnson, D. G., Myers, G. G.: Hydrocephalus as a complication of jugular catheterization during total parenteral nutrition. J. Pediatr. Surg. 10, 771-777 (1975)
References Puljic, MD New York Medical College 1249 Fifth Avenue New York, NY 10029, USA S.
1. Bering, E. A., Salibi, B.: Production of hydrocephalus by increased cephalic venous pressure. AMA Arch. Neurol. Psychiatry 10, 693-698 (1959)
Neuroradiology16, 76-78 (1978)
© by Springer-Verlag1978
Radiography of Hydrocephalus after Total Parenteral Nutrition S. Puljic, L. J. Newman, L. Heitlinger, M. Y. Chaudhary, E. Hiesiger, and D. Kotsilimbas The Departments of Pediatrics, Radiology,and Neurosurgery,New York MedicalCollege,New York, New York, USA
Summary. An infant with chronic diarrhea developed hydrocephalus following treatment with total parenteral nutrition (TPN) via jugular vein catheterization. Total parenteral nutrition is used when nutritional needs cannot be met adequately by oral alimentation. Serial computerized tomograms showed progression of communicating hydrocephalus. Superior sagittal sinograms demonstrated bilateral internal jugular vein occlusion with extensive venous eoUateralization. Lumboperitoneal shunt effectively decreased raised CSF pressure. A judicious approach to alternative venous routes for hyperalimentation is suggested. Radiographic delineation of communicating hydrocephalus by computerized tomography and superior sagittal sinography is presented.
Communicating hydrocephalus following bilateral jugular vein thrombosis as a result of hyperalimentation has recently become a focus of attention. Stewart et al. [6] reported .four cases in which the development of communicating hydrocephalus was related to the prolongation of total parenteral nutrition via jugular vein catheterization. However, there was no radiological evidence of venous occlusion. We report on a patient with hydrocephalus following jugular vein thrombosis. Both jugular veins were catheterized for the purpose of central parenteral alimentation. The importance of radiological investigation for diagnostic and prognostic purposes is emphasized.
Case Report A 4-month-old male infant presented with chronic diarrhea. He had been fed Similiac 20 ® until 2 weeks of age when he developed severe watery diarrhea. He was admitted to a community hospital in a state of moderate dehydration. The child was treated with intravenous
therapy to correct metabolic abnormalities and restore normal hydration. With reintroduction of oral alimentation, diarrhea recurred. His weight at 3 months was 3.6 kg. A diagnosis of chronic intractable diarrhea of infancy was made and central parenteral alimentation was started. A Silastic catheter was threaded into the right atrium from the right jugular vein and a solution of free amino acids (Freeamine®) and glucose was given. After 12 days' insertion of the catheter, the patient developed swelling about the face and right shoulder. The catheter was removed and inserted into the left internal jugular vein. After 7 days' insertion, swelling of the left side of the neck occurred. The rate of administration was then decreased, and oral alimentation with Pregestimil® was started. Since diarrhea worsened with oral feeding, the Pregestimil® was discontinued. Twenty-six days after insertion, the catheter in the left internal jugular vein was removed. We first saw the child when he was transferred to the Pediatric Nutrition Service at New York Medical College for further treatment. At this time the child, now 4 months of age, was well hydrated. The head circumference was 42 cm (50th percentile), weight was 4 kg (below 3rd percentile). On physical examination surgical scars of the neck, swelling of the neck, and puffiness of the eyelids were found. Neurological development w a s normal for his age. Results of laboratory studies revealed no fluid or metabolic abnormalities. A mild anemia was present. On an oral dietary regimen and intravenous replacement of continuing diarrheal losses, improvement ensued over a 3-week period and the child was discharged. Follow-up examinations at 5 months revealed a head circumference of 43 cm (between 50th and 75th percentile) and weight of 5.8 kg (3rd percentile). At 8 months the head circumference was 48 cm (97th percentile). A C T scan (Fig. la) showed communicating hydrocephalus of moderate degree. A ventrieuloperitoneal shunt was attempted, but the catheter was not placed in the ventricle. By 14 months of age, the head circumference was 53 cm. The anterior fontanelle was 0028-3940178/0016[0076/$01.00
S. Puljic et al.: Radiography of Hydrocephalusafter Total Parenteral Nutrition
77
Discussion
Fig. la. Initial CT scan at 8 months demonstrates moderate ventricular dilatation, widening of subarachnoid spaces especiallyinto the interhemispheric area. b. CT scan at 22 months shows mild ventricular dilatation and normal sulei
Fig. 2a. Lateral view of superior sagittal sinogram shows obstruction of internal jugular system with vertebral collateralization,b. Aiateroposteriorviewof superior sagittal sinogram shows obstruction of both jugular veins with venous eollaterization
open and bulging. The sutures had widened. A lumbar puncture was performed. The opening pressure was 290 mmH20; the closing pressure was 270 mmH20. A repeat CT scan showed progression of the hydrocephalus and cerebral edema. A carotid arteriogram to demonstrate a venous phase was not attempted; access to the carotid and femoral vessels was impeded by prior surgical explorations for venous cutdowns. A superior saglttal sinogram through 19-gauge butterfly needle (Fig. 2a, b) revealed obstruction of both internal jugular veins with extensive venous collateralization in the neck communicating with the superior vena cava. A lumboperitoneal shunt was inserted. During the ensuing 6 months the child developed normally. The head circumference remained 53.5 cm. The anterior fontanelle was no longer bulging and the sutures appeared normal. CT scans were almost normal (Fig. lb).
Communicating hydrocephalus is often acquired and may result from the impairment of resorption of cerebrospinal fluid at the level of arachnoid villi. Cerebrospihal fluid is absorbed into the venous system and any disturbance of the reabsorption process results in hydrocephalus. The mechanism by which a superior vena cava-like syndrome develops was clarified by Bering and Salibi [1] in studies in dogs where the entire cephalic venous outflow system was ligated, resulting in hydrocephalus. They postulated that i~ydrocephalus is due to the fact that increased venous pressure offsets the osmotic pressure in veins which in turn makes absorption of CSF difficult. In addition, increased pulsation of choroid plexuses contributes to the ventricular dilatation. Others [5] have shown that the collapse of the sagittal sinus, secondary to ventricular enlargement, further compromises venous outflow and decreases CSF absorption by the arachnoid villi. Possible compensatory mechanisms to relieve the hydrocephalus include the development of venous coUaterals in the emissary-external jugular systems and the vertebral plexus. This is brought about by the increased CSF pressure as well as the transventricular absorption of CSF. However, shunts which relieve this CSF pressure may also remove the stimulus for collateral development. Without shunting, ventricular expansion may lead to decompensated hydrocephalus [2, 4]. Haar and Miller [3] studied the problem of venous obstruction in hydrocephalic patients with dural sinus hypertension. They found that some of their patients had pseudotumor cerebri when the venous system was only partly occluded. In these cases, the ventricles were normal in size and the venous pressure was normal. Others had communicating hydrocephalus owing to bilateral venous outflow occlusion. In these cases, enlargement of the ventricles and increased intracranial venous pressure occurred. Our patient had features of both these types of cases. Central catheters were inserted into both internal jugular veins at different times for TPN. This resulted in a superior vena cava-like syndrome withventricular enlargement, cerebral edema, enlarged subarachnoid space, and dilatation of the cisterns. Perhaps this could have been avoided had the catheter been removed as the first signs of venous occlusion appeared. Because attempts to insert a ventriculoperitoneal shunt failed, the clinical signs of progressive hydrocephalus, i.e., increasing head circumference, bulging anterior fontanelle, and open suture lines, were persistent. The CSF pressure was exceedingly high (opening pressure 290 mmH20, closing pressure 270 mmH20). Serial CT scans demonstrated the increasing cerebral swelling and ventricular enlargement. Superior sagittal sinogram is an optional procedure to demonstrate jugular vein obstruction and venous collateralization. Manual injection of 15 cc of Renografin 60% through 19-gauge butterfly
78
S. Puljic et al.: Radiography of Hydrocephalus after Total Parenteral Nutrition
needle is sufficient to give excellent opacification. The procedure is fast, safe, easy, and well-tolerated. After a lumboperitonea[ shunt was inserted, the CSF pressure returned to normal; the anterial fontanelle became flattened. The rate o f head growth decreased. In the ensuing months the child developed normally. This report illustrates a complication of parenteral alimentation in infants with catheterization of the Cephalic venous outflow tracts. A judicious approach to alternative venous routes should be explored. The combined use of the computerized tomograms and the superior sagittal sinogram helped to delineate the type of hydrocephalus and the site o f venous obstruction. These diagnostic techniques showed the necessity for successful shunting in this patient.
2. Epstein, Z., Hochwald,G. M., Wald, A., Ransohoff, J.: Avoidance of shunt dependency in hydrocephalus. Dev. Med. Child Neurol. 17, 71-103 (1975) 3. Haar, E. L., Miller, C. A.: Hydrocephalus resulting from superior vena cava thrombosis in an infant. J. Neurosurg. 42, 597 to 601 (1975) 4. Hochwald, G. M., Epstein, F., Malhan, C., Ransohoff, J.: The relationship of compensated to decompensated hydrocephalus in the cat. J. Neurosurg. 39,694-697 (1973) 5. Shulman, K., Yarnell, R., Ransohoff, J.: Dural sinus pressure in normal and hydrocephalic dogs. Arch. Neurol. 81,575-580 (1964) 6. Stewart, D., Johnson, D. G., Myers, G. G.: Hydrocephalus as a complication of jugular catheterization during total parenteral nutrition. J. Pediatr. Surg. 10, 771-777 (1975)
References Puljic, MD New York Medical College 1249 Fifth Avenue New York, NY 10029, USA S.
1. Bering, E. A., Salibi, B.: Production of hydrocephalus by increased cephalic venous pressure. AMA Arch. Neurol. Psychiatry 10, 693-698 (1959)
