PSU Vol 03, 1994

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PEDIATRIC SURGERY UPDATE

VOLUME 03, 1994

VOLUME 03 NO 01 JULY 1994

Bile-Plug Syndrome

Define as a partial or complete intraluminal obstruction of the extrahepatic bile ducts caused by an abnormal, viscous, inspissated bile. Clinical presentation is th

jaundice. This condition is associated to: massive hemolysis from Rh or ABO incompatibility, cystic fibrosis, TPN, and ileal resections. Ultrasound findings are: d

or stone formation, dilated common bile duct with sludge (echogenic appearance), and no acoustic shadowing. DISIDA shows no intestinal excretion of the radiois

spontaneously. Those associated to worsening of direct hyperbilirubinemia and liver function tests should be managed surgically. Irrigation of bile ducts with salin

through the gallbladder is curative.

References

1- Lang EV, Pinckney LE: Spontaneous resolution of bile-plug syndrome. AJR Am J Roentgenol 156(6):1225-6, 1991

2- Brown DM: Bil e plug syndrome: successful management with a mucolytic agent. J Pediatr Surg 25 (3):351 -2, 1990

3- Mahr MA, Hugosson C, Nazer HM, Saad SA, Ali MA: Bile-plug syndrome. Pediatr Radiol 19(1):61-4, 1988

4- Pfeiffer WR, Robinson LH, Bals ara VJ: Sonographic features of bile plug syndrome. J Ultrasound Med 5(3):161-3, 1986

Gallstones

With the increase use of sonography in the work-up of abdominal pain, cholelithiasis is diagnosed more frequently in children. Gallstones occur as conse quence o

constituents. Two types are recognize: cholesterol and bilirubin. Those of cholesterol are caused by supersaturation of bile (lithogenic) by cholesterol overproduct

Bilirubin stones occur due to hemolysis (Sickle Cell, thalassemia) or bacterial infection of bile. Other etiologies include: Ascaris Lumbricoides infestation, drug-in

resection, TPN. etc. Gallbladder sludge is a clinical entity that when it persists can be a predisposing factor for cholelithiasis and cholecystitis. Management of sy

consists of laparoscopic cholecystectomy which has found to: reduce hospital stay, increase child return to normal activities , is well tolerated, low complication rat

procedure.

References

1- Riccabona M, Kerbl R, Schwinger W, Spork D, Millner M, Grubbauer HM: [Ceftriaxone-induced cholelithiasis--a harmless side-effect?]Klin Padiatr 205(6):421-3, 1993

2- Gerry F, Jaby O, Forbin C, Ebrad P: [Surgical treatment of cholelithiasis in children with sickle-cell anemia] Pediatrie (Bucur) 46(2):209-12, 1991

3- Schulman A: Intrahepatic biliary stones: imaging features and a possible relationship with ascaris lumbricoides. Clin Radiol 47(5):325-32, 1993

4- Bhattacharyya N, Wayne AS, Kevy SV, Shamberger RC: Perioperative management for cholecystectomy in sickle cell disease. J Pediatr Surg 28(1):72-5, 1993

5- Ware RE, Kinney TR, Casey JR, Pappas TN, Meyers WC: Laparoscopic cholecystectomy in young patients with sickle hemoglobinopathies. J Pediatr 120(1):58-61, 1992

6- Heubi JE, O'Connell NC, Setchell KD: Ileal resection/dysfunction in childhood predisposes to lithogenic bile only after puberty. Gastroenterology 103(2):636-40, 1992

7- Erdamar I, Avci G, Fuzun M, Harmancioglu O: Extracorporeal shockwave lithotripsy and litholytic therapy in cholelithiasis. Br J Surg 79(3):235-6, 1992

8- Reif S, Sloven DG, Lebenthal E: Gallstones i n children. Characterization by age, etiology, andoutcome [see comments] Am J Dis Child 145(1):105 -8, 1991

On HD Etiology

Hirschsprung's disease (HD) is characterized by lack of enteric ganglion cells, hyperplasia of abnormal nerve fibers and a non- propulsive, non-relaxing segment

etiology is attributed to a failure of cranio-caudal migration of parasympathetic neural crest cells to the distal bowel. A plausible explanation for the failure of rela

is a deficiency of enteric inhibitory nerves that mediates the relaxation phase of peristalsis. This nerves are intrinsic to the gut and are classify as non-adrenergic

oxide (NO) has recently been implicated as the neurotransmitter which mediates the relaxation of smooth muscle of the GI tract in HD. It's absence in aganglioni

failure of relaxation during peristalsis. Besides adhesions molecules (absent in aganglionic bowel) during early embryogenesis might restrict the neuro-ectoderma

contact between nerves and muscle cell (synaptogenesis) suggesting that developmental anomaly of innervated muscle and absent NO causes the spasticity char

References

1- Whalen TV Jr, Asch MJ: Report of two patients with hypertrophic pyloric stenosis and Hirschsprung's disease. Coincident or common etiology? Am Surg 51(8):480-1, 1985

2- Pass arge E: The genetics of Hirschsprung's disease. Evidence for heterogeneous etiology and a study of sixty-three families. N Engl J Med 276(3):138-43, 1967

3- Tam PK, Quint WG, van Velzen D: Hirschsprung's disease: a viral etiology? Pediatr Pathol 12(6):807-10, 1992

CF Prenatal Diagnosis

Cystic Fibrosis is an autosomal recessive lethal disease affecting 1 in 2000 live newborns. Is caused by different mutations in the cystic fibrosis transmembrane c

gene. Prenatal diagnosis is possible to couples of carriers with a one- fourth risk of having an affected newborn. Reliability of the test is 98%. The sample is take

week gestation) or amniocentesis. A DNA polymerase chain reaction analysis has shown a major mutation in the delta F508 position (is a three base pair deletion

protein without the amino acid phenylalanine at position 508) of the long arm of chromosome 7.

References

1- DeMarchi JM, Beaudet AL, Caskey CT, Richards CS: Experience of an academic reference laboratory using automation for analysis of cystic fibrosis mutations. Arch Pathol Lab Med 118(1):26-3

2- Mennie M, Compton M, Gilfillan A, Axton RA, Liston WA, Pullen I, Whyte D, Brock DJ: Prenatal screening for cystic fibrosis: attitudes and responses of participants. Clin Genet 44(2):102-6, 19

3- Ferec C, Verlingue C, Audrezet MP, Guillermit H, Quere I, Raguenes O, Mercier B: Prenatal diagnosis of cystic fibrosis in different European populations: application of denaturing gradient gel

8(5):341-50, 1 993

4- Denayer L, Evers-Kiebooms G, De Boeck K, Van den Berghe H: Reproductive decision making of aunts and uncles of a child with cystic fibrosis: genetic risk perception and attitudes toward carri

diagnosis . Am J Med Genet 44(1):104-11 , 1992

5- Mennie ME, Gilfillan A, Compton M, Curtis L, Liston WA, Pullen I, Whyte DA, Brock DJ: Prenatal screening for cystic fibrosis. Lancet 340(8813):214-6, 1992

VOL 03 NO 02 AUGUST 1994

Intestinal Neuronal Dysplasia

Intestinal Neuronal Dysplasia (IND) is a colonic motility disorder first described in 1971 by Meier-Ruge associated to characteristic histochemical changes of the

submucous & myenteric plexus with giant ganglia formation, isolated ganglion cells in lamina propia and muscularis mucosa, e levation of acetylcholinesterase in p

lamina propia and circular muscle, and myenteric plexus sympathetic hypoplastic innervation),also known as hyperganglionosis as sociate d to elevated acetylcholi

staining. The condition can occur in an isolated form (either localized to colon or disseminated throughout the bowel), or associated to other diseases such as Hirs

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neurofibromatosis, MEN type IIB, and anorectal malformations. It is estimated that 20-75% of HD cases have IND changes proximal to the aganglionic segmen

types of isolated IND have been described: Type A shows symptoms of abdominal distension, enterocolitis, bloody stools, intestinal spasticity in imaging studies (

less common and associate d with hypoplasia of sympathetic nerves. Type B is more frequent, symptoms are indistinguishable from that of HD, with chronic const

repeated episodes of bowel obstruction. Management depends on clinical situation; conservative for minor symptoms until neuronal maturation occurs around the

and resectional therapy for life threatening situations.

References

1- Lugo-Vicente HL: Neuronal Intestinal Dysplasia: A Role for S urgery? Boletin Asoc Med PR 87(3-4): 60-63 ,1995

Prenatal Cystic Hygroma

Cystic hygroma (CH) is an uncommon congenital lesion of the lymphatic system appearing as a multilocular fluid filled cavity most commonly in the back neck re

with exte nsive involvement of airway or vital s tructures . The e tiology is intrauterine failure of lymphatics to communicate with the venous sys tem. Prenatal diagn

first trimester of pregnancy as a huge neck tumor. Differential diagnosis includes teratomas, encephalocele, hemangiomas, etc. There is a strong correlation betw

syndrome (> 50%), structural defects (Noonan's syndrome) and chromosomic anomalies (13, 18, 21). Early diagnosis (< 30 wk gestation) is commonly associated

immune hydrops and dismal outcome (fetal death). Spontaneous regress ion is le ss likely but can explain webbed neck of Turner and Noonan's children. Prenatal

cytogenetic analysis: chorionic villous sampling, amniocentesis, or nuchal fluid cell obtained from the CH itself to determine fetal karyotype and provide counseli

diagnosis (>30 wks) should be delivered in tertiary center prepare to deal with dystocia and postnatal dyspnea of newborn. The airway should be secured before c

References

1- Anderson NG, Kennedy JC: Prognosis in fetal cystic hygroma. Aust N Z J Obstet Gynaecol 32(1):36-9, 1992

2- Iloki LH, Azika ME, Bouramoue V:[Prenatal diagnosis of cystic hygroma of the neck by echography. Case report. Review of the literature] Rev Fr Gynecol Obstet 87(1):42-4, 1992

3- Bernard P, Chabaud JJ, Le Guern H, Le Bris MJ, Boog G:[Cystic hygroma of the neck. Antenatal diagnosis, prognostic factors, management. 42 cases] J Gynecol Obstet Biol Reprod (Paris) 20(4

4- Pijpers L, Reuss A, Stewart PA, Wladimiroff JW, Sachs ES: Fetal cystic hygroma: prenatal diagnosis and management. Obstet Gynecol 72(2):223-4, 1988

5- Cohen MM, Schwartz S, Schwartz MF, Blitzer MG, Raffel LJ, Mullins-Keene CL, Sun CC, Blakemore KJ: Antenatal detection of cystic hygroma. Obstet Gynecol Surv 44(6):481-90, 1989

6- Abramowicz JS, Warsof SL, Doyle DL, Smith D, Levy DL: Congenital cystic hygroma of the neck diagnosed prenatally: outcome with normal and abnormal karyotype. Prenat Diagn 9(5):321-7, 19

GER and RS

Although a fundoplication is effective in controlling gastroesophageal reflux (GER), it could be less effective in curing respiratory symptoms (RS) such as aspirati

cough and wheezing attributed to reflux in children. The problem is documenting a cause -effe ct relationship between GER and RS. Objective parameters used are

reflux episodes during sleep by 18-24 hr ph-study), isotopic tracing in lung during milk scintigrams and sx preceded by a drop in ph. A ZMD > 4 min can be a relia

surgery will cure the RS. Reversible obstructive airway disease pts. are more prone to suffer from GER secondary to RS with less chances of being relieved by f

impaired child with RS has highest rate of failure. Medical tx and transgastric jejunostomy feeding are effective for poor operative risk pts.

References

1- Halpern LM, Jolley SG, Tunell WP, Johnson DG, Sterling CE: The mean duration of gastroesophageal reflux during sleep as an indicator of respiratory symptoms from gastroesophageal reflux i

26(6):686-90, 19 91

2- de Bli c J, Revillon Y, Scheinmann P: The rel ationship between gas troesophageal reflux and chronic res piratory diseas es. Clin Rev Allergy 8(4):427-41 , 199 0

3- Baris h CF, Wu WC, Castell DO: Respiratory complications of gas troesophageal reflux. Arch Intern Med 145(10):1882 -8, 198 5

4- Orenstein SR, Orenstein DM: Gastroesophageal reflux and respiratory disease in children [published erratum appears in J Pediatr 1988 Sep;113(3):578] J Pediatr 112(6):847-58, 1988

5- Padman R, Quijano R: Gastroesophageal reflux and recurrent/chronic pulmonary disease in infants and children. Del Med J 61(10):547-54, 1989

VOL 03 NO 03 SEPTEMBER 1994

TPNIC

TPN induced cholestatic (TPNIC) jaundice is an uncommon conjugated hyperbilirubinemia in infant which carries serious implications. Infectious, metabolic, and s

cholestasis should be rule-out. The incidence of TPNIC is around 30-50% of infants receiving prolonged TPN. Very low birth weight and prematures are more at

single etiologic factor has been identified, although large amounts of amino acids associated to low amounts of fat in the TPN solution can cause sludge and stone

The most important factor in the hepato-biliary dysfunction is the patient underlying disease state and severity of his illness. TPNIC is a predisposing factor for s

since it leads to impaired non-specific cellular immunity. Cholestasis is as sociated with intracranial hemorrhage, PDA, sepsis , and GI conditions requiring surgery

markedly elevated liver enzymes, no excretion of Tc labeled HIDA and increased GGT levels. Liver histology correlates with the chronologic progression of the

as follows: by 5 days of TPN you can find steatosis with prominent eosinophils in portal tracts, by 10 days there is canalicular cholestasis, by 21 days bile duct pro

fibrosis and after 5 months micronodular cirrhosis. Phenobarbital which increase bile flow has not been found beneficial in TPNIC. In most cases the degree of dy

reversible upon cessation of TPN.

References

1- Horsl en SP: Cholestasis in infancy: 1. Br J Hosp Med 50(11):674-7, 1993

2- Komura J, Yano H, Tanaka Y, Tsuru T: Increased incidence of cholestasis during total parenteral nutrition in children--factors affecting stone formation. Kurume Med J 40(1):7-11, 1993

3- Rintala R, Lindahl H, Pohjavuori M, Saxen H, Sariola H: Surgical treatment of intractable cholestasis associated with total parenteral nutrition in premature infants. J Pediatr Surg 28(5):716-9, 1

4- Nousia-Arvanitakis S, Angelopoulou-Sakadami N, Metroliou K: Complications associated with total parenteral nutrition in infants with short bowel syndrome. Hepatogastroenterology 39(2):169-7

5- Payne-James JJ, Silk DB: Hepatobiliary dysfunction associated with total parenteral nutrition. Dig Dis 9(2):106-24, 1991

6- Bell RL, Ferry GD, Smith EO, Shulman RJ, Christensen BL, Labarthe DR, Wills CA: Total parenteral nutrition-related cholestasis in infants. JPEN J Parenter Enteral Nutr 10(4):356-9, 1986

7- Merritt RJ: Cholestasis associated with total parenteral nutrition. J Pediatr Gastroenterol Nutr 5(1):9-22, 1986

8- Lilly JR, Sokol RJ: On the bile sludge syndrome or is total parenteral nutrition choles tasis a surgical disease? P ediatrics 76(6):992-3, 1985

GER and NI

Feeding disturbances , vomiting, anticonvulsant drug non-compliance, and recurrent chest infections in neurologically impaired (NI) children ofte n request the e ffo

improve growth, nutrition, quality of life and reduce se izure activity. Options are enteral tube fee ding, percutaneous e ndoscopic gas trostomy (PEG), and open gas

antireflux surgery (ARS). Whenever a NI child is referred for feeding gastrostomy a expeditious search for gastroesophageal reflux (GER) is achieved and ARS

higher rate of complication than in non-NI children (4:1). They include: wrap herniation into chest (entire or paraesophageal), disruption and recurrent preop GER

pneumonia are the most troublesome). The etiology of NI offers little help in predicting success of ARS. Theories explaining this high rate of failure are: supine p

incoordination, esophageal dysmotility, spasticity, seizures, delayed gastric emptying, chronic constipation and scoliosis (increase intrabdominal pressure). Not evthe presence of GER. Improving nutrition can decrease GER symptoms. PEG can help improve nutrition and serve as a screening tool for those pts. with continui

who will eventually need ARS. Ballantine permanent roux-en-y jejunostomy is another alternative in pts. with persistent feeding intolerance after ARS.

References

1- Pearl RH, Robie DK, Ein SH, Shandling B, Wesson DE, Superina R, Mctaggart K, Garcia VF, O'Connor, Filler RM: Complications of gastroesophageal antireflux surgery in neurologically impai

children. J Pediatr Surg 25(11):1169-73, 1990

2- Reyes AL, Cash AJ, Green SH, Booth IW: Gastrooesophageal reflux in children with cerebral palsy. Child Care Health Dev 19(2):109-18, 1993

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3- Glassman MS, Dozer AJ, Newman LJ: Gastroesophageal reflux in neurologically impaired children: perioperative evaluation and management. South Med J 85(3):289-92, 1992

WT and HA

In 1967 hyaluronic acid (HA), a glycosaminoglycan is detected in the serum and urine of a patient with a Wilm's Tumor (WT). HA has also been found in children

hyperviscosity , suppress the formation of humoral precipitating antibodies to certain major classes of proteins (interfering with elicitation of a complete antibody

platelet dysfunction presenting as coagulopathy suggesting Acquire von Willebrand disease. The group from UCSF School of Medicine postulated that since the f

circulating HA stimulating activity and WT is an embryonal tumor, HA could serve as a WT tumor marker. A cooperative study started in 1990 gathered data for

urine levels in WT pts are high preop, decrease with tumor resection and persist high with tumor persistence or relapse (APSA 94).

References

1- Longaker MT, Adzick NS, Harrison MR, Stern R: A new tumor marker for Wilm's tumor called hyaluronic

acid-stimulating activity (HASA) [letter] J Pediatr S urg 25(9):1015, 19902- Kumar S, West DC, Ponting JM: Hyaluronic acid and childhood renal tumors [letter; comment] J Natl Cancer Inst 82(11):973-4, 1990

3- Longaker MT, Adzick NS, S adigh D, Hendin B, Stair SE, Duncan BW, Harrison MR, Spendlove R, Stern R: Hyaluronic acid-stimulating activity in the pathophysiology of Wilms' tumors [s ee co

82(2):135-9, 1 990

4- Hopwood JJ, Dorfman A: Glycosaminoglycan synthesis by Wilms' tumor. Pediatr Res 12(1):52-6, 1978

5- Morse BS,Nussbaum M: The detection of hyaluronic acid in the serum and urine of a patient with nephroblastoma. Am J Med 42(6):996-1002, 1967

6- American Pediatric Surgi cal Ass ociation (APSA) 1994 Meeting

VOL 03 NO 04 OCTOBER 1994

Acute Pancreatitis

Acute pancreatitis (AP) is unusual in the pediatric patient, can affect all age groups and should be considered in children presenting with acute abdominal complai

clinical course less severe. The three most common etiological factors are: trauma, drug-induced, and biliary tract disorders. Other factors to consider are: infecti

adenovirus), metabolic (branched-chain organic acidemias), structural defects (anomalous union of pancreatico-biliary ductal system), and hereditary. Blunt abdo

cause (20-30%)of AP by crushing the fixed organ between the spine. Drugs associated to the development of AP are: steroids, L-asparaginase, valproic acid, aceis treatment of choice). Biliary disorders related to AP are gallstone and choledochal cysts by causing transient ductal obstruction. Most common complaint of chi

pain. Diagnosis is confirmed with elevated amylase/lipase in serum and urine (lipase is more specific since pancreas is major source). Imaging studies of utility ar

The use of ERCP in previously idiopathic cases of AP have increased the yield of diagnosing anomalous pancreatico-biliary junctional defects. Management duri

with IV therapy, NG decompression, NPO (to decrease acid stimulation and prevent secretin release), and nutritional (TPN). Surgery is rarely required except co

and pseudocyst formation.

References

1- Simon HK, Muehlberg A, Linak is JG: Serum amylase determinations in pediatric patients presenting to the ED with acute abdominal pain or trauma. Am J Emerg Med 12(3 ):292-5, 1994

2- Kahler SG, Sherwood WG, Woolf D, Lawless ST, Zaritsk y A, Bonham J, Taylor CJ, Clarke JT, Durie P, Leonard JV: Pancreatitis in patients with organic acidemias. J Pediatr 124 (2):239-43, 19

3- Kozarek RA, Christie D, Barclay G: Endoscopic therapy of pancreatitis in the pediatric population. Gastrointest Endosc 39(5):665-9, 1993

4- Mori K, Nagakawa T, Ohta T, Nakano T, Kayahara M, Aki yama T, Kanno M, Ueno K, Konishi I, Izumi R, et al: Pancreatitis and anomalous union of the pancreaticobili ary ductal system in childhoo

28(1):67-71, 1 993

5- Winchester M: Pancreatitis in children. Gastroenterol Nurs 15(3):125-8. 19 92

6- Moir CR, Konzen KM, Perrault j: Surgical therapy and long-term follow-up of childhood hereditary pancreatitis. J Pediatr Surg 27(3):282-6, 1992; discussion 286-7

7- Mader TJ, McHugh TP: Acute pancreatitis in children. Pediatr Emerg Care 8(3):157-61, 1992

Water in Neonates

Water represents 70-80% of body weight of normal neonates and premature babies respectively. Total body water (TBW) varies inversely with fat content (prem

deposits). TBW is distributed into 1/3 e xtracellular (ECF) and 2/3 intracellular (ICF) fluids compartments. The ECF is compose d of plasma volume (4-5% body we

transcellular fluids. There is a change in body water upon entrance of the fetus to his new extrauterine existence; A gradual decrease in TBW and ECF, and an in

is interrupted with a premature birth. Inappropriate IV therapy can lead to persistent PDA, bronchopulmonary dysplasia, NEC, and intraventricular hemorrhage.

from lung (1/3) and skin (2/3). Transepithelial (skin) water loss is the major component and decrease s with increas e in postnatal age. Radiant warmers and photot

losses . Ne wborns produce a urine output of 2 cc/kg/hr to clear the osmotic solute load at an osmolality of 250 mOsm/kg. Their kidney have a low glomerular filtra

ability (limited urea in medullary interstitium) and are therefore less tolerant to dehydration. Electrolytes requirement of full-term: Na 2meq/kg/day, K 2 meq/kg/

100cc/kg/24 hrs for the first 10 kg of weight.

References

1- Hellers tein S: Fluid and electrolytes: clinical aspects. Pediatr Rev 14(3):103-15, 19 93

2- Boineau FG, Lewy JE: Estimation of parenteral fluid requirements. Pediatr Clin North Am 37(2):257-64, 1990

3- S tatter MB: Fluids and electrolytes in infants and children. Semin Pediatr Surg 1(3):208-11, 1992

4- Shaffer SG, Weis mann DN: Fluid requirements in the preterm infant. Clin Perinatol 19(1):233-50, 19925- De Bruin WJ, Greenwald BM, Notterman DA: Fluid resuscitation in pediatrics. Crit Care Clin 8(2):423-38, 1992

6- Hammarlund K: Neonatal adaptation of fluid balance. J Perinat Med 19 Suppl 1:80-5, 1991

Hepatoblastoma

Hepatoblastoma (HB) is the most common primary malignant neoplasm of the liver in children mostly s een in males less than four year of age. Diagnostic work-u

Scan) objective is predicting resectability and tumor extension. Diagnostic laparotomy will decide resectability. Markers associated to this tumor are: alpha-fetop

glutamyltransferase II. Only reliable chances of cure is surgical excision although half are unresectable at dx. Unresectable tumors can be managed with preop c

preop chemotx are: progressive disease, increase morbidity, post-op complications, and toxicity. Advantages are: decrease in tumor size, covert three-fourth case

extent of surgery is not decreased. Tumor necrosis is more extensive in pt. receiving preop chemotx. Osteoid present in tumors after chemotx may represent an i

to maturate and differentiate. Diploid tumors on DNA flow cytometry show a bette r prognosis.

References

1- von Schweinitz D, Burger D, Mildenberger H: Is laparatomy the first step in treatment of childhood liver tumors?--The experience from the German Cooperative Pediatric Liver Tumor Study HB-8

1994

2- S axena R, Leake JL, Shafford EA, Davenport M, Mowat AP, Pr itchard J, Mieli-Vergani G, Howard ER, Spitz L, Malone M, et al: Chemotherapy effects on hepatoblastoma. A histological study. Am1993

3- Conrad RJ, Gribbin D, Walker NI, Ong TH: Combined cystic teratoma and hepatoblastoma of the liver. Cancer 72(10):2910-3 , 199 3

4- Tagge EP. Tagge DU. Reyes J. Tzakis A. Iwatsuki S. Starzl TE. Wiener ES: Resection, including transplantation, for hepatoblastoma and hepatocellular carcinoma: impact on survival. J Pediatr S

297

5- King DR, Ortega J, Campbell J , Haas J, Ablin A, Lloyd D, Newman K, Quinn J, Krailo M, Feusner J, e t al: The surg ical management of children with incompletely resected hepatic cancer is facil

Pediatr Surg 26(9):1074-80, 1991; discussion 1080-1

6- Gauthier F, Valayer J, Thai BL, Sinico M, Kalifa C: Hepatoblastoma and hepatocarcinoma in children: analysis of a series of 29 cases. J Pediatr Surg 21(5):424-9, 1986

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VOL 03 NO 05 NOVEMBER 1994

CVC Sepsis

Central Venous Catheter (CVC) placement has improved care and survival of sick infants. Catheter sepsis with an incidence of 10-30% remains the most commo

insertion. Defined as an apparent clinical infection in a patient with a CVC proven by positive blood culture when no other source can explain it. Principal isolated

negative staphylococci (CONS). These organisms are associated to foreign body placement (V-P shunts, CVC, etc.) due to their adherents properties. Although c

can occur during insertion or subsequent care, catheter hub contamination is the primary site of entry of organism with secondary colonization of the tip. The mec

cleaned entry site. Factors which increase the incidence CVC sepsis are: younger age, primary diagnosis, use of TPN (lipid emulsions), multiple catheter use, thro

formation. Management of definite CVC sepsis consists of antibiotics or removal. The use of urokinase and antibiotics can effectively and safely manage these e

90% of infected catheter salvage. Persistent multiple organisms or fungal sepsis will need catheter removal. A dilemma is to leave a possible infected or removeAOLC (Acridine-orange leucocyte cytospin) tes t is specific and sensitive in rapidly (one hour) distinguishing cathete r septicemia when compared with NBT and C

include: skin disinfection of catheter exit site with two 10-second application of povidone swabs every 24 hour, and occlusion with a sterile semipermeable dressin

added to TPN solutions has eliminated CONS-CVC sepsis and increased useful catheter life. Staff training in caring for CVC will reduce the incidence of CVC rel

References

1- Tan TQ, Musser JM, Shulman RJ, Mason EO Jr, Mahoney DH Jr, Kaplan SL: Molecular epidemiology of coagulase-negative Staphylococcus blood isolates from neonates with persistent bacterem

catheter infections. J Infect Dis 169(6):1393-7, 1994

2- Soong WJ, Hwang B: Percutaneous central venous catheterization: five year experiment in a neonatal intensive care unit. Acta Paediatr Sin 34(5):356-66, 1993

3- Bansal V, Strauss A, Gyepes M,Kanchanapoom V: Central line perforation associated with Staphylococcus epidermidis infection. J Pediatr Surg 28(7):894-7, 1993

4- Malathi I, Millar MR, Leeming JP, Hedges A, Marlow N: Skin disinfection in preterm infants. Arch Dis Child 69(3 Spec No):312-6, 1993

5- Rushforth JA, Hoy CM, Kite P, Puntis JW: Rapid diagnosis of central venous catheter sepsis. Lancet 342(8868):402-3, 19 93

6- Jones GR, Konsl er GK, Dunaway RP, Lacey SR, Azizk han RG: Prospective analysis of urokinase in the treatment of catheter sepsis in pediatric hematology-oncology patients. J Pediatr Surg 2 8(

7- Salzman MB, Isenberg HD, Shapiro JF, Lipsitz PJ, Rubin LG: A prospective study of the catheter hub as the portal of entry for microorganisms causing catheter-related sepsis in neonates. J Infec

8- Garland JS, Dunne WM Jr, Havens P, Hintermeyer M, Bozzette MA, Wincek J, Bromberger T, Seavers M: Peripheral intravenous catheter complications in critically ill children: a prospective s

50, 1992

9- Puntis JW, Holden CE, Smallman S, Finkel Y, George RH, Booth IW: Staff training: a key factor in reducing intravascular catheter sepsis. Arch Dis Child 66(3):335-7, 1991

Bezoars

Bezoars are rare foreign body concretions formed in the s tomach and small bowel composed mainly of hair (tricho), ve getable matter (phyto) or milk curds (lacto).

children, 6-10 years old, with bizarre appetite (trichophagia) and emotional disturbances. Originally the mass forms in the stomach and can move to the small bow

exte nsion or total translocation. Diagnosis can be confirmed by UGIS, CT-Scan or endoscopy. The child can develop an asymptomatic palpable abdominal mass, p

perforation. Other children will reduce intake and develop weight loss. Predisposing conditions to bezoar formations are: gastric dymotility and decreased acidity.

mechanical or pulsating jet of water fragmentation via the endoscope, operative extraction, shock-wave lithotripsy (ESWL) with subsequent evacuation, or dissol

proteolytic enzymes (papain, acetylcysteine, cellulase). With ESWL the shock wave pressure needed is less than half used for urolithiasis cases.

References

1- Santiago-Snchez C, Garau-Daz P,Lugo-Vicente HL: Trichobezoar in a 11-year old girl : A Case Report. Boletin Asoc Med PR Vol 88 (1-3): 8-11 , 1996

2- Sharma V, Sharma ID: Intestinal trichobezoar with perforation in a child. J Pediatr Surg 27(4):518-9, 1992

3- Wadlington WB, Rose M, Holcomb GW Jr.: Complications of trichobezoars : a 30-year experience. Southern Medical Journal. 85(10):1020-2 , 1992

4- Rao PL, Mitra SK, Pathak IC: Trichobezoars in children. International Surgery 66(1 ):63-5, 19 815- Vergara Rodriguez J, Sarinana Natera C: Bezoars in childhood. [Spanish] Boletin Medico Del Hospital Infantil De Mexico. [JC:ag0] 34(6):1205-13 , 197 7

Cytokines

Cytokines (CK) are proteins produced by our own cells (macrophages , lymphocytes , mast ce lls, fibroblasts, etc.) which modulate the acute phase of inflammation

neoplastic growth. They play a key role in control of hemopoiesis and immunity. Characterized so far: tumor necrosis factor (TNF), interleukin (IL) 1 to 6, recentl

Recombinant technologies have made them available. CK enhances neonatal myeloid progenitor proliferation, modulates neonatal bone marrow neutrophil storag

induces peripheral neutrophilia and protects against the increase mortality associated with bacterial sepsis. Early postoperative increases in plasma IL-6 and IL-8

response of neonates to surgery with an exaggerated response during postop complications. IL-6 is responsible for the acute phase response, and IL-8 with mark

References

1- Chang M, Suen Y, Lee SM, Baly D, Buzby JS, Knoppel E, Wolpe S, Cairo MS: Transforming growth factor-beta 1, macrophage inflammatory protein-1 alpha, and interleukin-8 gene expression is

compared with adult mononuclear cells. Blood 84(1):118-24, 1994

2- Mathew P, Crist WM, Furman WL: The use of cytokines in children. Curr Opin Pediatr 6(1):58-67, 1994

3- Hayward A, Cosyns M: Proliferative and cytokine responses by human newborn T cells stimulated with staphylococcal enterotoxin B. Pediatr Res 35(3):293-8, 1994

4- Wolf SS, Cohen A: Express ion of cytokines and their receptors by human thymocytes and thymic stromal cells . Immunology 77(3):362-8, 19 925- Hettmannsperger U, Detmar M, Owsianowski M, Tenorio S, Kammler HJ, Orfanos CE: Cytokine-stimulated human dermal microvascular endothelial cel ls produce interleukin 6 --inhibition by h

calcitriol. J Invest Dermatol 99(5):531-6, 1992

6- Splawski JB, Lipsky PE: Cytokine regulation of immunoglobulin secretion by neonatal lymphocytes. J Clin Invest 88(3):967-77, 1991

7- Parkman R: Cytokines and T lymphocytes in pediatrics. J Pediatr 118(3):S21-3, 1991

VOL 03 NO 06 DECEMBER 1994

SCT

Sacrococcygeal teratoma (SCT) is the most common extragonadal germ cell tumor in neonates with an incidence of one in 30-40,000 live births. Three-fourth are

large, firm or more commonly cystic masse s that arise from the anterior surface of the sacrum or coccyx, protruding and forming a large exte rnal mass. Histology

three germ cell layers. SCT is class ified as: mature, immature, or malignant (endodermal sinus) and produces alpha-feto protein (AFP). Prenatal sonographic diag

tumor size greater than the biparietal diameter of the fetus, rapid tumor growth, deve lopment of placentomegaly, polyhydramnios and hydrops. Large tumors sho

section to avoid dystocia or tumor rupture. Management consist of total tumor resection with coccyx (recurrence is associated with leaving coccyx in place). Everbe regarded as potentially malignant. Malignant or immature SCT with elevated AFP after surgical resection will benefit from adjuvant chemotherapy. Survival is

tumors, but less than 80% for malignant cases. Follow-up should consist of (1) meticulous physical exam every 3-6 months for first three years, (2) serial AFP det

fecal/urodynamic functional studies. Long term F/U has found a 40% incidence of fecal and urinary impairment as sociated to e ither tumor compress ion of pelvic s

References

1- Inoue M, Kubota A, Hasegawa T, Hata S, Takahashi E, Kawahara H, Suehara N, Okada A: Antenatal diagnos is of sacrococcygeal teratoma with hydrops fetalis ; a case report. Eur J Pediatr Surg 4(

2- Evans MJ, Danielian PJ, Gray ES: Sacrococcygeal teratoma: a case of mis taken identity. Pediatr Radiol 24(1):52-3 , 1994

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3- Teitelbaum D, Teich S, Cassidy S, Karp M, Cooney D, Besner G: Highly vascularized sacrococcygeal teratoma: description of this atypical variant and its operative management. J Pediatr Surg 29

4- Rintala R, Lahdenne P, Lindahl H, Siimes M, Heikinheimo M:Anorectal function in adults operated for a benign sacrococcygeal teratoma. J Pediatr Surg 28(9):1165-7, 1993

5- Bilik R. Shandling B. Pope M.Thorner P. Weitzman S. Ein SH: Malignant benign neonatal sacrococcygeal teratoma. J Pediatr Surg 28(9):1158-60, 1993

6- Liu KK. Lee KH. Ku KW: Sacrococcygeal teratoma in children: a diagnostic challenge. Aust N Z J Surg 64(2):102-5, 1994

7- Boemers TM, van Gool JD, de Jong TP, Bax KM: Lower urinary tract dysfunction in children with benign sacrococcygeal teratoma. J Urol 151(1):174-6, 1994

8- S chropp KP

AU - Lobe TE, Rao B, Mutabagani K, Kay GA, Gilchris t BF, Philippe PG, Boles ET Jr: Sacrococcygeal teratoma: the experience of four decades. J Pediatr Surg 27(8):1075-8, 1992; discus sion 1078-

Grave's

Hyperthyroidism in children is caused by a non-immune autonomous adenoma (rare), hyperfunctional nodular goiter (less frequent), or more commonly diffuse im

(Grave's Dise ase). Grave's children manifest initial neurologic symptoms and tachycardia. Management consist of: (1) medical- antithyroid drugs, hormone subst

(2) surgical excision, or (3) radio-iodine therapy (not routine use d due to possible induction of malignancy). After two years of antithyroid drug therapy hyperthyro

case s. The only factor predicting remission with drug tx is absence of ophthalmopathy. Main disadvantages of antithyroid drugs are the need for prolonged treat

Indications for surgery are: poor compliance with drug tx, recurrent hyperthyroidism after prolonged (two years) drug tx, side effects to drugs (neutropenia, vasc

unpleasant or obstructive symptomatic goiter. Before surgical therapy clinical and biochemical euthyroidism should be obtained. Extent of resection is debatable

expertise, exte nt of recurrent hyperthyroidism (8-12% incidence), and rate of hypothyroidism complications. Most prefe r subtotal thyroidectomy (retaining 6-10

gland removal. Surgical complications are: recurrent nerve paralysis, temporary or permanent hypoparathyroidism, and post-op thyrotoxic crisis. Ophthalmopath

disappears when euthyroidism is obtained. Lymphocytic infiltration if found in three-fourths of glands removed.

References

1- Mariotti S, Caturegli P, Barbesino G, Ceccarelli C, Lippi F, Marino M, Manetti L, Martino E,Pinchera A: [Radiometabolic therapy of the autonomous thyroid nodule] Minerva Endocrinol 18(4):155

2- Rajasoorya C: Examining the therapeutic options in hyperthyroidism--a personal perspective.Ann Acad Med Singapore 22(4):617-23, 1993

3- Sills IN, Horlick MN, Rapaport R: Inappropriate suppression of thyrotropin during medical treatment of Graves disease in childhood. J Pediatr 121(2):206-9, 1992

4- Csaky G, Balazs G, Bako G, Ilyes I, Kalman K, Szabo J: Late results of thyroid surgery for hyperthyroidism performed in childhood. Prog Pediatr Surg 26:31-40, 1991

5- Rauh V, Kujath HP, Reimers C, Hocht B: Indications, surgical treatment and after-care in juvenile hyperthyroidism. Prog Pediatr Surg 26:28-30, 1991

6- Ladurner D, Riccabona G: Surgical aspects of diseases of the thyroid gland in childhood. Prog Pediatr Surg 26:15-20, 1991

LH-RH

Gonadotropin releasing hormone (LH-RH) is a decapeptide that when used as nasal spray will cause descend of one-third of unilateral palpable undescended test

subsequent use of Human Chorionic Gonadotropin. Treatment is more effective the younger the age of the boys and the near is the testis to the scrotum. LH-RH

therapy of testicular non-descent because is non-invasive, has good response in younger age groups, and limited side-effects. The number of germ cells per tubule

patient who responds to LH-RH. Contraindications for hormonal therapy are: (1) coexistent inguinal hernia or hydrocele tes tis, and (2) previous inguinal operatio

have poor response to hormonal therapy.

References

1- Christiansen P, Muller JR, Buhl SB, Hansen OR, Hobolth N, Jacobsen BB, Jorgensen PI, Kastrup KW, Nielsen K, Nielsen LB, et al:[Cryptorchism. Treatment with human chorionic gonadotropi

hormone?] Ugeskr Laeger 155(41):3287-90, 1993

2- Thorup J, Cortes D, Nielsen OH: Clinical and histopathological evaluation of operated maldescended testes after LH-RH treatment. Eur J Pediatr 152 Suppl 2:S37, 1993

3- Waldschmidt J, Doede T, Vygen I: The results of 9 years of experience with a combined treatment with LH-RH and HCG for cryptorchidism.Eur J Pediatr 152 Suppl 2:S34-6, 1993

4- Frick J: LHRH and cryptorchidism. Eur J Pediatr 152 Suppl 2:S28-30, 199 3

5- Christiansen P, Muller J, Buhl S, Hansen OR, Hobolth N, Jacobsen BB, Jorgensen PH, Kastrup KW, Nielsen K, Nielsen LB, et al: Hormonal treatment of cryptorchidism--hCG or GnRH--a multic

81(8):605-8, 1 992

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