Mediastinal pancreatic pseudocyst in infancy

Mediastinal pancreatic pseudocyst in infancy

Volume 91 Number 3 Brief clinical and laboratory observations procedures, t h o u g h relatively easy to p e r f o r m , c a n lead to complications...

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Volume 91 Number 3

Brief clinical and laboratory observations

procedures, t h o u g h relatively easy to p e r f o r m , c a n lead to complications.

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REFERENCES 1. Bucci G, Scalamandre A, Savignoni PG, Orzalesi M, and Mendicine M: Crib-side sampling of blood from the radial artery, Pediatrics 37:497, 1966. 2. Adams JM, Rudolph AJ: The use of indwelling radial artery catheters in neonates, Pediatrics 55:261, 1975. 3. Matthews JI, and Gibbons RB: Embolization complicating radial artery punctures, Ann Jntern Med 75:87, 1971. 4. Bedford RF, and Wollman H: Complications of percutaneous radial artery cannulation, Anesthesiology 38:228, 1973. 5. Mathieu A, Dalton B, Fischer JE, and Kumar A: Expanding aneurysm of the radial artery after frequent puncture, Anesthesiology 38:401, 1973. 6. Taylor N: Carpal tunnel syndrome, Am J Phy Med 50:192, 1971. 7. Koenigsberger MR, Curtin J, and Lovelace RE: Motor conduction velocities as a measure of gestational age in premature infants: a study of multiple births, twins, triplets, and quadruplets, Neurology 20:381, 1970. 8. Koenigsberger MR, Driscoll JM Jr, and Curtin J: Peripheral neuropathy in infants receiving intravenous hyperali-

Mediastinal pancreatic pseudocyst in infancy Robert E. Mallard, M.D.,* C.A. Stilwell, M.D., James A. O'Neill, Jr., M.D., and David T. Karzon, M.D., Nashville, Tenn. R E C U R R E N T HEMORRHAGIC PLEURAL EFFUSIONS associated with a mediastinal mass d u r i n g infancy presented a challenging p r o b l e m with multiple diagnostic possibilities. This report presents the first case o f mediastina! extension of a pancreatic pseudocyst identified in a n infant.

CASE R E P O R T A 10-month-old infant was referred to the Children's Hospital of Vanderbilt University for evaluation of recurrent hemorrhagic pleural effusions.

From the Departments of Pediatric Surgery and Pediatrics, Children's Hospital of Vanderbilt University. *Reprint address: Vanderbilt University Hospital, Nashville, TN 37232.

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mentation, proceedings of the Child Neurology Society, Hamilton, Ontario, Canada, October, 1975. Thomas JE, and Lambert EH: Ulnar nerve conduction velocity and H-reflex in infants and children, J Appl Physiol 15:1, 1960. Wagner AL, and Buchtal F: Motor and sensory conduction in infancy and childhood reappraisal, Dev Med Child Neurol 14:189, 1972. Schulte FJ, Michaelis R, Linke I, and Nolte R: Motor nerve conduction velocity in term, preterm and small for dates newborn infants, Pediatrics 42:17, 1968. Wigger HJ, Bransilver BR, and Blanc WA: Thrombosis due to catheterization in infants and children, J PEDIATR 76:1, 1970. Nabseth D, and Jones JE: Gangrene of the lower extremities of infants after femoral venipuncture, N Engl J Med 268:1003, ~1963. Macon WL, and Futrell JW: Median-nerve neuropathy after percutaneons puncture of the brachial artery in patients receiving anticoagulants, N Engl J Med 288:1396, 1973. Starreveld E, and Ashenhurst EM: Bilateral carpal tunnel syndrome in childhood, Neurology 25:234, 1975. Patten BM: Neuropathy induced by hemorrhage, Arch Neurol 21:381, 1969.

The patient had had feeding difficulties since birth and was an irritable infant. Hospitalization at age seven months followed the acute onset of respiratory distress. Chest roentgenograms revealed a massive left pleural effusion and thoracentesis yielded hemorrhagic pleural fluid. Fluid (100 to 250 ml) was aspirated from left and right pleural spaces on multiple occasions. Results of cultures for bacteria, mycobacteria, and fungi were negative. Tuberculin and histoplasmin skin tests were negative as were multiple cultures of blood, bone marrow, and pleural fluid. Serum immunoglobulin concentrations were normal. Antibiotics were given without benefit. Evaluation for neoplastic disease was unrevealing. Examinations of pleural fluid cytology, bone marrow, intravenous pyelogram, upper gastrointestinal roentgenograms, skeletal survey, lung tomograms, and urinary vanillylmandelic acid and catecholamine excretion were negative. An exploratory laparotomy was performed at age eight months for a right upper quadrant mass. Numerous inflammatory adhesions were found and a "mesenteric cyst" was removed from the right transverse mesocolon. Open lung, pleural, and mediastinal node biopsies were not helpful to diagnosis. Removal of the pleural effusions was regularly associated with marked clinical improvement and disappearance of respiratory distress. The infant maintained weight gain and appetite throughout the illness except during periods of respiratory distress. Continued recurrence of bilateral hemorrhagic pleural effusions prompted referral. The infant was irritable, with height at the 50% and weight at the 3% level. There was tachypnea with intercostal retractions and decreased breath sounds throughout the right hemithorax.

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The Journal of Pediatrics September 1977 DISCUSSION

Fig. 1. Anteroposterior and oblique views after injection of radiographic contrast media reveals the anterior mediastinal cyst and its communication beneath the diaphragm to the region of the head of the pancreas. Heart sounds were decreased in intensity. The abdomen was tense and distended without palpable masses. Initial laboratory studies included: white blood cell count, 19,000/ram ~ with normal differential, hemoglobin 11.8 gm/dl, erythrocyte sedimentation rate 25 ram/hour, SMA-18 was unrevealing. Serum amylase value was 200 Somogyi units. A chest roentgenogram showed pleural thickening bilaterally, enlarged cardiac silhouette, and a right anterior mediastinal mass not previously identified. Liver-spleen scan and abdominal ultrasound examination were normal. Efforts to withdraw pleural fluid failed. An echocardiogram demonstrated fluid anterior and posterior to the heart. A diagnostic tap of the anterior space yielded 90 ml of serosanguinous fluid, with glucose concentration of 54 mg/dl, protein of 970 mg/dl, and amylase of 8,885 Somogyi units. Cultures and cytology of the fluid were negative. Cardiac catheterization was unremarkable. The fluid filled anterior mediasainal space was injected with radiographic contrast material, revealing a large cystic mass in the anterior mediastinum extending into the right chest and beneath the anterior left hemidiaphragm to the region of the head of the pancreas (Fig. 1). Exploratory laparotomy confirmed the preoperative diagnosis of pancreatic pseudocyst with mediastinal extension. A tubular, cystic mass originated in the head of the pancreas which communicated with the mediastinal pseudocyst through the anterior left diaphragm. The pseudocyst was divided at the diaphragm and a Roux-en-y jejunocystostomy constructed over the head of the pancreas. An operative pancreatogram demonstrated another cyst in the head of the pancreas which was made to communicate with the main cyst. A sphincterotomy was performed. The mediastinal pseudocyst was separated from its pancreatic connection and drained externally by marsupialization. Postoperative serum amylase values fell to less than 15 Somogyi units. The child is growing well and without difficulties 15 months postoperatively.

Pancreatic pseudocysts are u n c o m m o n in children. Mediastinal extension of a pancreatic pseudocyst is even more unusual, with only 15 cases reported in all ages. 1-4 Two mediastinal pancreatic pseudocysts have been reported in childhood: one in a 10-year-old girl? the other in a 15-year-old boy. ~' Several reviews discuss abdominal pancreatic pseudocysts in childrens -~' A b d o m i n a l trauma is responsible for approximately 60%; stones, malignancy, and m u m p s are implicated in several instances; and 30% arise without demonstrable cause. The battered child syndrome should be considered when no etiology is found. In our case, no etiology was determined. Whatever the insult to the pancreas, it has been proposed that hemorrhagic necrosis and pancreatic enzyme exudation lead to an inflammatory reaction which is followed by fibrous connective tissue proliferation. This leads to encapsulation and formation of a pseudocyst contiguous with the pancreass The pseudocyst usually remains within the lesser sac, but may dissect into the gastrocolic omentum, the transverse mesocolon, or through the diaphragm. Mediastinal extension occurred through the aortic hiatus in 40% (6/15) of reported cases, through the esophageal hiatus in 60% (9/15) and by direct extension through the diaphragm in 7% (1/15). Abdominal pancreatic pseudocysts are associated with abdominal pain (68%), abdominal mass (64%), vomiting (52%), and fever (24%)? Mediastinal extension may lead to respiratory distress. Pleural effusion occurred in only one of 73 abdominal pancreatic pseudocysts in children. Pleural effusion occurred, however, in 10 of 15 reported cases (67%) of mediastinal pseudocysts. Amylase concentration in pleural and pseudocyst fluid is the ~ost specific diagnostic guide. Serum amylase concentrations are elevated in 88% of children with pseudocysts, and urine amylase determinations increase this yield. ~ Pseudocyst fluid amylase levels are three to 50 times that of the serum level. In the present case the cyst fluid amylase was 8,885 and the serum amylase 200 units. Pleural fluid amylase greater than 200 units, or greater than serum levels, are diagnostic of effusions secondary to pancreatic disease.'" Surgical m a n a g e m e n t of pancreatic pseudocysts has varied." Aspiration, total excision, external drainage by catheter or marsupialization, and internal drainage into the gastrointestinal tract have been employed. Of 15 patients with mediastinal pseudocysts reported to date, ten have had internal drainage procedures and only one had recurrence. To our knowledge, this is the first reported occurrence of mediastinal extension of a pancreatic pseudocyst in an

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Br&f clinical and laboratory observations

infant. Although the child has remained well since operation, long-term evaluation will be important. The authors gratefully acknowledge the contribution of Dr. Felix Line and Dr. John Maddox, Knoxville, Tennessee, who provided important background information and referred this patient to the Children's Hospital of Vanderbilt University. REFERENCES 1. Dewey GC, and Clark RE: Middle mediastinal and retrogastric mass, Chest 69:97, 1976. 2. Christensen NM, Demling R, and Mathewson C: Unusual manifestations of pancreatic pseudocysts and their surgical management, Am J Surg 130:199, 1975. 3. Jaffe BM, Ferguson TB, Holtz S, and Shields JB: Mediastinal pancreatic pseudocysts, Am J Surg 124:600, 1972.

False negative meconium test results in screening for cystic fibrosis Nila Desai, M.D., and Sanda Nousia-Arvanitakis, M.D.,* Kansas City, Kan.

THE VALUE of the Boehringer-Mannheim m e c o n i u m test for routine screening for cystic fibrosis has been questioned recently because of the high proportion of false negative results? This report presents our observations on the validity of the B-M m e c o n i u m test. PATIENTS

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4. Anderson W J, Skinner DB, Zuidema GD, and Cameron JL: Chronic pancreatic pleural effusions, Surg Gynecol Obstet 137:827, 1973. 5. Galligan JJ, and Williams H J: Pancreatic pseudocysts in childhood, Am J Dis Child 112:479, 1966. 6. Laird CA, and Clagett OT: Mediastinal pseudocyst of the pancreas in a child: report of a case, Surgery 60:465, 1966. 7. Miller RE: Pancreatic pseudocysts in infants and children, Arch Surg 89:517, 1964. 8. Cooney DR, and Grosfeld JL: Operative management of pancreatic pseudocysts in infants and children, Ann Surg 182:590, 1975. 9. Wool G, and Goldring D: Pseudocyst of the pancreas, J PEDIATR70:586, 1967. 10. Kaye MD: Pleuropulmonary complications of pancreatitis, Thorax 23:297, 1968.

provided by the manufacturer. A hematest was also carried out on each sample. At 4 to 8 weeks, determination of sweat electrolyte concentrations was performed on each infant; sweat was obtained by pilocarpine iontophoresis. Concentrations of sweat chloride and sodium above 60 and 70 mEq/1, respectively, were considered confirmatory of CF. Fecal fat and coefficient of absorption were determined in two infants. Steatorrhea was present when excretion of fat was greater than 18 g m / 7 2 hours and coefficient of absorption was less than 80%. Assay of fecal chymotrypsin ~ was also carried out in the two infants at 11 to 12 months of age. Fecal chymotrypsin values lower than 150 ~ g / g m of stool were considered suggestive of exocrine pancreatic insufficiency.

Abbreviations used B-M: Boehringer-Mannheim CF: cystic fibrosis

AND METHODS

Specimens of meconium were obtained from 318 neonates delivered at Menorah Medical Center, Kansas City, Mo. The samples were stored at 4 ~ C and transferred to the CF Laboratory at the University of Kansas Medical Center within 48 hours. The B-M m e c o n i u m test was performed and interpreted according to the directions

From the Department of Pediatrics, University of Kansas Medical Center. Supported by a Grantfrom the Cystic Fibrosis Foundation. *Reprint address: Department of Pediatrics, University of Kansas Medical Center, 39th St. at Rainbow Blvd, Kansas City, KS 66103.

RESULTS The B-M test was negative in the meconiums of 317 newborn infants (Table I). One neonate had a positive test. Hematest was negative in the meconiums of all infants. Subsequent sweat tests on each infant were negative in 316 cases including that of the infant who had had a positive B-M m e c o n i u m test. The a m o u n t of sweat collected was 210 _+ 110 mg (mean +__ SD). The concentrations of sweat chloride and sodium were 11 ___ 6 mEq/1 and 19 +_ 9 m E q / l , respectively. The infant who was found to have a positive B-M m e c o n i u m test was well at three months. The sweat test was repeated; the sweat