PSU Vol 15, 2000

7/30/2019 PSU Vol 15, 2000

http://slidepdf.com/reader/full/psu-vol-15-2000 1/6

6/18/12 PSU Vol 15, 2000

1/6home.coqui.net/titolugo/PSU15.htm#1562

PEDIATRIC SURGERY UPDATE ©

VOLUME 15, 2000

Volume 15 No 01 JULY 2000

Overwhelming Post-Splenectomy Infections

Splenectomy impairs the immune response to bacterial infections. Such impaired immunologic functions include: formation of antibodies , deficiency of opsonizatio

deficiency in bacterial clearing and tuftsin deficient phagocytosis. Overwhelming post-splenectomy infection (OPSI) refers to a constellation of fast-developing sy

hypotension, rigor, bacteremia, leucocytosis) that leads to death in patients that have undergo removal of the spleen. Mortality rates after OPSI is established ar

blood cultures grow encapsulated organisms (pneumococcus, meningococcus, hemophilus, etc.). The vulnerability of OPSI is greates t within the first two years aft

persists throughout life. The clinical appearance of OPSI can go from a mild event to death from sepsis with pulmonary complications as the most common morbid

identified after spleen removal for Hodgkin and trauma. Immunization against pneumococcus, H. Influenza and meningococcus should be given to all children who

these are the most common organisms associated with OPSI. In the elective situation the vaccine should be given two weeks prior to removal of the spleen. In set

given as soon as possible, Though several studies have found better functional antibody responses with delayed (14-day) vaccination in the setting of trauma we

vaccine as soon as possible until well-randomized trials are done.

References:

1- Jugenbu rg M, Hadd ock G, Freedman MH, Ford-Jones L, Ein SH: The morbidity and mortality of p ediatric splenec tomy: does prophy laxis make a difference? J Pediatr Surg 34(7):1064-7, 1999

2- Camel JE, Kim KS, Tchejeyan GH, Mahour GH: Efficacy o f pas sive immunot herapy in experimental pos tsp lenectomy s eps is du e to Haemophilus influenzae type B.J Pediatr Surg 28(11):1441-4, 1993

3- Green JB, Shackford SR, Sise MJ, Powell RW: Post sp lenectomy sepsis in pediatric patients following s plenectomy for trau ma: a proposal for a multi-institutional st udy. J Pediatr Surg 21(12):1084-6, 194- Hays DM, Ternbe rg JL, Chen TT, Sullivan MP, Tefft M, Fung F, Gilchrist G, Fryer C, Gehan EA: Posts plenectomy seps is and ot her complications following staging laparot omy for Hodgkin's diseas e i

21(7):628-32, 1986

5- Reihner E, Brismar B: Manag ement of s plenic trauma--chang ing con cepts . Eur J Emerg Med 2(1):47-51, 1995 6- Shatz DV; Schinsky MF; Pais LB; Romero-Steiner S; Kirton OC; Carlone, GM: Immune responses of splenectomized trauma patients to the 23-valent pneumoccal polysaccharide vaccine at 1 versus 7

Trauma 44(5):760-5, 1998

7- Schreiber MA, Pusateri AE, Veit BC, Smiley RA, Morrison CA, Harris RA: Timing of vaccination does not affect antibody response or survival after pneumococcal challenge in splenectomized rats. J

8- Caplan ES, Boltansky H, Snyder MJ, Rooney J, Hoyt NJ, Schiffman G,Cowley RA: Response of traumatized splenectomized patients to immediate vaccination with polyvalent pneumococcal vaccine. J

Esophageal Hernias

Two types of esophageal hernia recognized are the hiatal and paraesophageal hernia. Diagnosis is made radiologically always and in a number of patients endosc

(HH) refers to herniation of the stomach to the chest through the esophageal hiatus. The lower esophageal sphincter also moves. It can consist of a small transito

(minor) up to an upside-down intrathoracic stomach (major). HH ge nerally develops due to a congenital, traumatic or iatrogenic factor. Most disappear by the age

HH can lead to peptic esophagitis from Gastroesophageal reflux. Repair of HH is determined by the pathology of its associated reflux (causing failure to thrive, e

respiratory symptoms) or the presence of the stomach in the thoracic cavity. In the paraesophageal hernia (PH) variety the stomach migrates to the chest and the

stays in its normal anatomic position. PH is a frequent problem after antireflux operations in patients without posterior crural repair. Small PH can be observed.

appearance of symptoms (reflux, gastric obstruction, bleeding, infarction or perforation) the PH should be repaired. The incidence of PH has increased with the a

fundoplication.

References:

1- Bettex M, Oesch I: The Hiatus Hernia Saga. Ups and Downs in Gastroesophageal Reflux: Past, Present, and Future Perspectives. J Pediatr Surg 18(6): 670-680, 1983

2- Avans ino JR, Lorenz ML, Hendrickso n M, Jo lley SG: Characterization an d management o f paraes ophag eal hernias in children after an tireflux operation. J Pediatr Surg 34(11):1610-4, 1999

3- Kim SH; Hendren WH; Don ahoe PK: Gastroes ophag eal reflux and hiatus hernia in children: experience with 70 cases . J Pediatr Surg 15(4):443-51, 1980

4- Bernhard UA, Shmerling DH: Follow-up examinations of cons ervatively an d surgically treated children with hiatus hernia. Prog Ped iatr Surg 18:118-31, 1985

5- Alrabeeah A, Giacomantonio M, Gillis DA: Paraeso phageal hernia after Nissen fundop lication: a real complication in pediatric patients . J Pediatr Surg Au g;23(8):766-8, 1988 6- Basso N, De Leo A, Genco A, Rosato P, Rea S, Spaziani E, Primavera A: 360 degrees laparoscopic fundoplication with tension-free hiatoplasty in the treatment of symptomatic gastroesophageal reflux

2000

Rectal Duplication

Rectal duplications are very rare encompassing 5% of all GI duplications. They can be cys tic or tubular (hindgut), small or involve a significant portion of the pro

Most are cystic arising in a retrorectal position and 90% do not communicate with the rectum. Presentation depends on size (mass effect), fistulization (drainage o

anus or a fistula is a frequent presenting sign), infection, the presence of ectopic gastric mucosa (causing ulceration & bleeding), prolapse, bladder outlet obstruct

degeneration (adenocarcinoma). Epithelial lining of the duplication is usually colonic, other types being squamous, epithelium, gas tric mucosa or urothelial. Bariu

and MRI are helpful in localizing the anatomy and extent. M anagement of the duplication depends on location and size. Surgical excision through a transanal, tra

sagittal approach is warrant in retrorectal cysts . Anterior duplications or those associated with a genitourinary malformation require a laparotomy. High index of s

delay and multiple operations. Complete excision is curative.

References:

1- La Quaglia MP, Feins N, Eraklis A , Hendren WH: Rectal Duplications . J Pediatr Surg 25(9): 980-984, 1990

2- Rajah S, Ramanujam TM, Anas SR, et al: Duplication o f the rec tum: report of fou r cases and rev iew of the literature. Ped iatr Surg Int 13: 373-376, 1998

3- Poenaru KA, Sobo leski D, Hurlbut D, Kamai I: Anterior Rectal Duplication: A Diagnos tic Challenge. J Ped iatr Surg 35(4): 613-614, 2000

4- Okur H, Kerkin E, Zo rludemir U, Olcay I: Tubular Dup lication of t he Hindgu t with Genitourinary Anomalies. J Pediatr Surg 27(9): 1239-1240, 1992

5- Gibson TC, Edwards JM, Shafiq S: Carcinoma arising in a rec tal duplication cy st. Br J Surg 73(5):377, 1986

6- Rauch MK, Martin EL, Cromie WJ: Rectal duplication as a cause of neonatal bladder outlet obstruction and hydronephrosis. J Urol 149(5):1085-6, 1993

7- Mboyo A, Monek O, Massicot R, Martin L, Destuynder O, Lemouel A, Aubert D: Cystic rectal duplication: a rare cause of neonatal intestinal obstruction. Pediatr Surg Int 12(5-6):452-4, 1997 8- Delarue A, Garcia-Meric P, Martin C, Piguet C, Andre N, Galli G, Guys JM: Anten atal rupt ure of a diverticular rect al duplication with neona tal perineal fistu lization. Ped iatr Surg Int 13(4):288-9, 1998

7/30/2019 PSU Vol 15, 2000


6/18/12 PSU Vol 15, 2000


Volume 15 No 02 AUGUST 2000

Carcinoid Syndrome

The carcinoid syndrome (fascial flushing, diarrhea, tricuspid regurgitation, pulmonic stenosis, valvular fibrosis and wheezing) is the result of serotonin overproduc

Carcinoid tumors arise from enterochromaffin cells (APUD ce lls from the neural crests ), occur in virtually every organ, could be multiple, metastatic and associat

Patients are diagnosed biochemically from increased urinary excretion of 5-hydroxyindoleacetic acid (5-HIAA). Platelet serotonin levels are more sensitive for de

secrete small amounts of serotonin. Jejunum-ileum, bronchus and appendix are the most common sites of origin. Carcinoid of the appendix is the most common ne

childhood. Metastasis to liver of midgut carcinoids produce the syndrome. Tumors greater than 2 cm are more prone to metastasis needing aggressive surgical m

and I-131 MIBG are use ful in dete rmination of location and extent of s ome carcinoid tumors, particularly those of midgut origin. A positive scan may predict the

to control symptoms of hormonal hypersecretion. Scans provide localization of the primary tumor that should be widely excised including lymph nodes. Higher sur

patients with midgut lesions who undergo intra abdominal debulking procedures excluding the liver. For single liver les ion rese ction is justified, otherwise with muartery ligation or embolization has been tried. Symptomatic metastasis should be managed with Octreotide. Prognosis is associated with the presence of liver met

development and level of tumor markers (chromogranin A).

References:

1- Hoberock TR, Knutso n CO, Polk HC Jr: Clinical aspects of invas ive carcinoid tu mors. South Med J 68(1):33-7, 1975

2- Hanson MW, Feldman JM, Blinder RA, Moore JO, Coleman RE: Carcinoid tumors: iodine-131 MIBG scintigraphy. Radiology 172(3):699-703, 1989

3- Feldman JM: Carcinoid tumors and the carcinoid s yndrome. Curr Probl Su rg 26(12):835-85, 1989

4- Soreide O, Berstad T, Bakka A, Sch rumpf E, Hanssen LE, Engh V, Bergan A, Flatmark A: Surgical treatment as a principle in patients with advanced ab dominal carcinoid tumors. Surge ry 111(1):48-54, 1

5- Janson ET, Holmberg L, Stridsberg M, Erikss on B, Theodors so n E, Wilander E, Oberg K: Carcinoid tumors: analysis of prog nos tic factors an d su rvival in 301 patients from a referral center. Ann Onco

6- Moertel CL, Weiland LH, Telander RL: Carcinoid t umor of th e ap pendix in the first two decades of life. J Pediatr Su rg 25(10):1073-5, 1990

7- Lamberts SW, Bakker WH, Reubi JC, Krenning EP: Somatost atin-recepto r imaging in the localization o f end ocrine tu mors. N Engl J Med 323(18):1246-9, 1990

Left Hypoplastic Colon Syndrome

Colonic obstruction in the newborn child could be the result of necrotizing enterocolitis, atresia, meconium plug syndrome, duplication cyst, Hirschsprung disease

syndrome. The left (small) hypoplastic colon syndrome (LHCS) is a very rare cause of colonic obstruction identified in newborns with characteristic roentgenogra

those of Hirschsprung's disease. Manifesting in the first 24-48 hours of life, LHCS is a functional disturbance related to immaturity of the intrinsic innervation of

common in low birth weight neonates or of diabetic mothers. Intestinal perforation, sepsis, hypoglycemia and death may occur. The diagnosis is suggested in a ba

of the left colon is small with a transitional zone at the splenic flexure. Management consists of hypoglycemia correction, antibiotics, nasogastric decompression a

babies the obstruction clears in 48-72 hours. When the clinical diagnosis is not readily apparent a rectal biopsy and sweat chloride test should be done to different

Hirschsprung disease and cystic fibrosis respectively. The narrowed left colon remains narrow in follow-up.

References:

1- Woodh urst WB, Kliman MR: Neonatal small left colon sy ndrome: report of two cas es. A m Surg 42(7):479-81, 1976

2- Davis W S, Campbell JB: Neonatal s mall left colon syn drome. Occurrence in asymptomatic infants of diabet ic mothers. Am J Dis Child 129(9):1024-7, 1975

3- Stewart DR, Nixon GW, Jo hns on DG, Condo n VR: Neonatal s mall left colon sy ndrome. An n Su rg 186(6):741-5, 1977

4- al-Salem AH, Khwaja S, Wo od BP: Radiological case of th e month. Neonatal s mall left colon syn drome. Am J Dis Child 144(11):1273-4, 1990 5- Philippart AI, Reed JO, Georges on KE: Neonatal small left colon syndrome: Intramural not intraluminal obst ruction. J Ped iatr Surg 10: 733, 1975

Beckwith-Wiedemann Syndrome

The Beckwith-Wiedemann Syndrome (BWS), first described in 1964, is characterized by the presence of macrosomia (gigantism), macroglossia, omphalocele and

lobules of the external ear. One of the more frequent metabolic changes is transient neonatal hypoglycemia, the result of pancreas cell hyperplasia. Inheritance o

uncertain. Most cases are sporadic, but a number of familial cases have been reported. BWS is associated with a predisposition to embryonal tumors, most comm

abnormalities found in these tumors affect the same chromosome region (11p15), which has been implicated in the etiology of BWS. Routine abdominal ultrasoun

up to the age of eight years is recommended for children with BWS.

References:

1- Wiedemann HR: Complexe malformatif familial avec hernie o mbilicale et macrogloss ie--un " syn drome nou veau" ? J Genet Hum 13:223, 1964

2- Engstro m W, Lindham S, Schofield P: Wiedemann-Beckwith syndrome. Eur J Pediatr 147(5):450-7, 1988 3- Lodeiro JG, Byers JW 3d, Chuipek S, Feinstein SJ: Prenata l diagnos is and perinatal management of the Beckwith-Wiedeman sy ndrome: a cas e and review. Am J Perinatol 6(4):446-9, 1989

4- Weksberg R, Squire JA: Molecular biology of Beckwith-Wiedemann syn drome. Med Ped iatr Oncol 27(5):462-9, 1996

5- Li M, Squire JA, W eksberg R: Molecular genet ics of Beckwith-Wiedemann syn drome. Curr Opin Pediatr 9(6):623-9, 1997

6- Steenman M, Westerveld A, Mannens M: Genetics of Beckwith-Wiedemann syndrome-associated tumors: common genetic pathways. Genes Chromosomes Cancer 28(1):1-13, 2000

7-Lugo-Vicente HL: Molecular Biology an d Genetics affecting Pediatric Solid Tumors. Bol Asoc Med PR (in press ).

Volume 15 No 03 SEPTEMBER 2000

Askin Tumor

Askin tumor (synonyms are primitive neuroectodermal tumor or Ewing's sarcoma) is a malignant small round cell tumor of mesenchymal origin affecting the thora

children and young adults with a tendency to recur locally. The rib is the most common site of primary tumor development. Establishing an accurate preoperative

difficult. Microscopy and immunohistological stain of the specific marker - neuron-specific enolase, is essential. CT scan is valuable for evaluating tumor extensio

chemotherapy and assessing recurrence after surgery, but can overes timate pleural, lung or diaphragmatic infiltration. MRI can dete rmine ches t wall muscle andNeither is adequate for adjacent lung invasion. Bone, bone marrow and lung are the most frequent sites of metastasis. Treatment includes radical surgical resecti

tissue), neoadjuvant (local control of disease is critical) and adjuvant chemotherapy plus radiation. Surgical resection, with en bloc removal of involved structures

provides excellent local control of malignant chest wall tumors. Human dura, prostethic material (Gortex, Marlex, Vicryl) and myocutaneous flaps have been use

with Askin tumors treated with aggressive pre-resection chemotherapy have smaller tumors to resect (less than 100 cc by volume) with improved survival. Overa

7/30/2019 PSU Vol 15, 2000


6/18/12 PSU Vol 15, 2000


References:

1- Dang NC, Siegel SE, Phillips JD: Malignant ches t wall tumors in children and youn g ad ults. J Pediatr Su rg 34(12):1773-8, 1999

2- Sawin RS, Conrad EU 3rd, Park JR, Waldhausen JH: Preresection chemotherap y improves surv ival for children with Askin tumors. Arch Surg 131(8):877-80, 1996

3- Walton JM, Bass J, Sambey E, Rubin SZ: Use of human dura in pediatric chest wall reconstruction after tumor resection. J Pediatr Surg 29(9):1189-91, 1994

4- Shamberger RC, Tarbell NJ, Perez-Atayd e A R, Grier HE: Malignan t s mall round cell tumor (Ewing's -PNET) of the ches t wall in ch ildren. J Ped iatr Surg 29(2):179-84, 1994

5- Bourque M D, Di Lorenzo M, Collin PP, Russ o P, Laberge JM, Mo ir C: Malignant small-cell tumor of th e th oracopu lmonary region: 'Askin tumor'. J Pediatr Surg 24(10):1079-83, 1989

6- Shamberger RC, Grier HE, Weins tein HJ, Perez-Atayd e AR, Ta rbell NJ: Ches t wall tumors in infancy an d ch ildhood. Cancer 15;63(4):774-85, 1989

7- Parikh PM, Charak BS, Banavali SD, Advani SH, Saikia TK, Gopal R, Borges AM, Chinoy RF, Desai PB: Treatment o f Askin Rosai tumor--need for a more aggres sive ap proach. J Surg Onco l 39(2):126-8

8- Grosfeld JL, Rescorla FJ, West KW, Vane DW, DeRosa GP, Provisor AJ, Weetman R: Chest wall resection and reconstruction for malignant conditions in childhood. J Pediatr Surg 23(7):667-73, 1988

9- Askin FB, Rosai J, Sibley RK, Dehner LP, McAlister WH: Malignant small cell tumor of the thoracopulmonary region in childhood: a distinctive clinicopathologic entity of uncertain histogenesis. Can

Gastric Duplication

Gastric duplications cysts account for less than 5% of all enteric duplications (the rarest form). As a duplication it is attached to its origin, has a well developed s

epithelial lining. Prenatal ultrasound finding of a cys t with peristaltic activity within the right upper quadrant of the fe tal abdomen s uggest the diagnosis. The most

duplication cyst is the greater curvature. Most are closed spherical cysts. Most cases are diagnosed during the first two years of life and are more common in fe

is an abdominal mass with vomiting. Complications reported consist of recurrent pancreatitis, hemorraghe, perforation, peritonitis, torsion and malignant change i

studies, US or CT-Scan suggests the diagnosis. Management of a gastric duplication cyst is surgical excision that can be accomplished laparoscopically. Gastric

pancreatic tissue can be found in the cyst wall.

References:

1- Bidwell JK, Nelson A: Prenatal ultrasonic diagnosis of congenital duplication of the stomach. J Ultrasound Med 5(10):589-91, 1986

2- Sieunarine K, Manmohansingh E: Gastric duplication cyst presenting as an acute abdomen in a child. J Pediatr Surg 24(11):1152, 1989

3- Bajpai M, Mat hur M, Dup lications of the alimentary tract: clues to t he missing links. J Pediatr Surg 29(10):1361-5, 1994

4- Blais C, Masse S: Preoperative ultrasound diagnosis of a gastric duplication cyst with ectopic pancreas in a child. J Pediatr Surg 30(9):1384-6, 1995

5- Koumanidou C, Montemarano H, Vakaki M, Pitsoulakis G, Savvidou D, Kakavakis K: Perforation of multiple gast ric duplication cys ts: diagn os is by son ograph y. Eur Radiol 9(8):1675-7, 1999

Bile Duct Rhabdomyosarcoma

The botryoid variety of embryonal Rhabdomyosarcoma (RMS) is the most common tumor of bile ducts presenting during early life. Peak incidence at three to fo

predominance. The tumor is characterized by multiple polypoid grape-like projections into the lumen of the common bile duct with plate-like thickening of the com

characterized by a high risk of local recurrence to adjacent lymph nodes and a low risk of remote metastasis. Obstructive jaundice, cachexia, pain and abdominal

presentation, often with fever and hepatomegaly. Attribution of these symptoms to hepatitis commonly delays definitive treatment. Other times the preoperative

choledochal cyst. US defines the relationship of the tumor with portal vessels and biliary tract while CT-Scan and MRI determine operability. Aggressive surgery

adjuvant therapies (chemotherapy and radiotherapy) appears to provide the bes t chance for a longer survival. Intra-operative cholangiography is a valuable tech

of biliary tree obstruction and verifying a functioning drainage procedure. The prognosis is poor and death is usually due to the effe cts of local invasion by the tu

References:

1- Taira Y, Nakayama I, Moriuchi A, Takahara O, Ito T, Tsuchiya R, Hirano T, Matsushita T: Sarcoma botryoides arising from the biliary tract of children. A case report with review of the literature. Acta

2- Lack EE, Perez-Atayde A R, Schus ter SR: Botryoid rhab domyosarcoma of t he b iliary trac t. Am J Su rg Path ol 5(7):643-52, 1981

3- Martinez-F LA, Haase GM, Koep LJ; Akers DR: Rhabdomyos arcoma of the biliary tree: th e cas e for agg ress ive su rgery. J Ped iatr Surg 17(5):508-11, 1982

4- Ruymann FB, Raney RB Jr, Crist WM, Lawrence W Jr: Rhabdomyosarcoma of the biliary tree in childhood. A report from the Intergroup Rhabdomyosarcoma Study. Cancer 56(3):575-81, 1985

5- Geoffray A, Couanet D, Montagne JP, Leclere J: Ultrasonography and computed tomography for diagnosis and follow-up of biliary duct rhabdomyosarcomas in children. Pediatr Radiol 17(2):127-31, 1

6- von d er Oelsnitz G, Spaar HJ, Lieber T, Mun chow B, Booss D: Embryona l rhabdomyos arcoma of the common bile duct. Eur J Pediatr Su rg 1(3):161-5, 1991

7- Sanz N, de M ingo L, Florez F, Rollan V: Rhabdomyos arcoma of th e biliary tree. Ped iatr Surg Int 12(2-3):200-1, 1997

8- Balkan E, Kiristioglu I, Gurpinar A, Sinmaz K, Ozkan T, Dogruyol H: Rhabdomyosarcoma of the biliary tree. Turk J Pediatr 41(2):245-8, 1999

Volume 15 No 4 OCTOBER 2000

Chylothorax

Effusion of lymph (chyle) into the pleural cavity is known as chylothorax. Chyle is cle ar-milky fluid with an elevated total protein and albumin leve l, a s pecific grav

presence of WBC with lymphocyte predominance (80%), and ele vated triglyceride (chylomicrons). In children is a potentially life-threatening disorder that has pr

nutritional (hypoalbuminemia), electrolyte (hyponatremia) and immunologic (lymphopenia, hypogammaglobulinemia, T-cell depletion) effects. Chylothorax has a c

lymphangiomatosis), acquired or idiopathic origin. Acquired chylothorax is most commonly found; the result of a direct lesion of the thoracic duct or lymphatic ves

central venous cathete rs or chest tubes insertions), during cardiac surgery, mediastinal malignancy (neuroblastoma) or infection, repair of a diaphragmatic hernia

vena cava obstruction (thrombosis). Initial management consists of: 1- chest tube drainage after failed thoracentesis (pleural space tamponade), 2- medium-chain

for a week (lymphatic decompression), 3- TPN if chylothorax increases or persists. More protracted course (4 week medical tx) will require surgery to locate and

lymphatics, ligate the thoracic duct, do chemical pleurodesis or place a pleuroperitoneal shunt. Those associated with venous obstruction or increase right sided c

volume, persist longer and are more difficult to manage.

References:

1- Puntis JW , Roberts KD, Handy D: How shou ld chyloth orax be managed? Arch Dis Child 62(6):593-6, 1987

2- Jalili F: Medium-chain triglycerides and to tal parent eral nutrition in th e management o f infants with congen ital chylothorax. South Med J 80(10):1290-3, 1987

3- Easa D, Balaraman V, Ash K, Thompson B, Boychuk R: Congenital chylotho rax and mediastinal neurob lastoma. J Pediatr Su rg 26(1):96-8, 1991

4- Le Coultre C, Oberhans li I, Mossaz A, Bugmann P, Faidut ti B, Belli DC: Postope rative chy lothorax in children: differences between vascular and traumatic origin. J Pediatr Surg 26(5):519-23, 1991

5- Allen EM, van Heeckeren DW, Spector M L, Blumer JL: Management of nu tritional and infectious complications of p os toperat ive chyloth orax in children. J Pediatr Surg 26(10):1169-74, 1991

6- van Straaten HL, Gerards LJ, Krediet TG: Chylothorax in the neona tal period. Eur J Pediatr 152(1):2-5, 1993

7- Bond SJ, Guzzetta PC, Snyd er ML, Randolph JG: Management o f pediatric po sto perative ch ylothorax. Ann Thorac Su rg 56(3):469-72, 1993

8- Kavvadia V, Greenoug h A, Davenpo rt M, Karani J, Nicolaides KH: Chylothorax after repair of con genital diaphrag matic hernia--risk facto rs an d morbidity. J Pediatr Surg 33(3):500-2, 1998

9- Engum SA, Rescorla FJ, West KW, Scherer LR 3rd: The use of pleuroperitoneal shunts in the management of persistent chylothorax in infants. J Pediatr Surg 34(2):286-90, 1999

10- Beghetti M, La Scala G, Belli D, Bugmann P: Etiology and management o f ped iatric chyloth orax. J Pediatr 136(5):653-8, 2000

7/30/2019 PSU Vol 15, 2000


6/18/12 PSU Vol 15, 2000


Multiple Endocrine Neoplasia Type 1

Multiple endocrine neoplasia type 1 (MEN-1) is an autosomal dominant disorder characterized by the combined occurrence of parathyroid, pancreatic islet and a

single inherited locus on chromosome 11, band q13, cause s MEN-1. Primary hyperparathyroidism (HPT) is the most common les ion of MEN-1. The albumin corre

normal, but the ionized calcium and PT hormone is elevated. Once elevated the patient develops insidious complication from hypercalcemia (pancreatitis, peptic u

weakness, neuropsychiatry disorders and nephrolithiasis). Surgical management of primary HPT in MEN-1 is controversial. The salient features of HPT in MEN

universal occurrence of multiglandular disease , an operative failure rate because of failure of both to identify all four glands and to perform a radical resection, a

recurrent disease , sometimes cause by supernumerary or ectopic gland involvement. Surgical principles should be (1) identification of all four glands, (2) subtotal

facilitate possible reoperation, and (3) excis ion of supernumerary thymic glands. Extirpation of a s ingle gland as a general primary procedure is inadequate causi

patients with primary HPT and MEN syndrome have multiple abnormal parathyroid glands, two populations of patients exist; one population has solitary or doubl

is uncommon, whereas the other population of patients has hyperplasia and persistent or recurrent disease is common.

References:

1- Trump D, Farren B, Wo oding C, et al: Clinical stud ies o f multiple end ocrine neoplasia type 1 (MEN1). QJM 89(9):653-69, 1996

2- Kraimps JL, Duh QY, Demeure M, Clark OH: Hyperparat hyroidism in multiple endocrine neop lasia syndrome. Surge ry. 112(6):1080-6, 1992

3- Thakker RV, Bouloux P, Wooding C, et al: Association of p arathyroid tumors in multiple endocrine n eoplas ia type 1 with los s o f alleles on chromosome 11. N Engl J Med 321(4):218-24, 1989

4- Tonelli F, Spini S, Tommasi M, Gabbrielli G: Intraoperative parathormone measurement in patients with multiple endocrine neoplasia type I syndrome and hyperparathyroidism. World J Surg 24(5):556-

5- O'Riordain DS, O'Brien T, Grant CS, et al: Surgical management of p rimary hy perparath yroidism in multiple en docrine n eoplas ia types 1 and 2. Surgery 114(6):1031-7, 1993

6- Hellman P, Skogseid B, Juh lin C, et al: Findings and long -term results of parat hyroid s urgery in multiple endoc rine neoplas ia type 1. World J Surg 16(4):718-22, 1992

Ranula

The word ranula comes from the Latin: rana, frog. Ranula is a large sessile cyst of the sublingual salivary gland in the floor of the mouth under the tongue. The le

sized cys t to one side or the other of the frenulum, or an enormous blue-gray translucent swelling that fills the mouth and cause respiratory problems. Two ranula

superficial, epithelial lined cyst resulting from ductal obstruction, and a cervical pse udocyst without epithelial lining resulting from extravasation of saliva (plungin

tissue planes of the neck and appear as a neck mass. In both cases management consists of excision of the thin-walled sac and sublingual gland if possible, or ma

cavity.

References:

1- Quick CA, Lowell SH: Ranula an d th e s ublingual s alivary glands . Arch Otolaryngol 103(7):397-400, 1977

2- Parekh D, Stewart M, Jos eph C, Lawson HH: Plunging ranula: a report of three case s and rev iew of the literature. Br J Surg 74(4):307-9, 1987

3- de Viss cher JG, van d er Wal KG, de Vogel PL: The plunging ranula. Patho genes is, diagno sis and management. J Craniomaxillofac Surg 17(4):182-5, 1989

4-Baurmash HD: Marsupialization fo r treatment of oral ran ula: a second look at t he p rocedure. J Oral Maxillofac Surg 50(12):1274-9, 1992

5- Morton RP, Bartley JR: Simple su blingual ranulas: pa thog enes is and management. J Otolaryng ol 24(4):253-4, 1995

Volume 15 No 5 NOVEMBER 2000

Hepatic Hemangioendothelioma

Hepatic hemangioendothelioma (HHE) is a rare, benign tumor that appears during the first six-months of life. Considered the most common vascular tumor of the

with a high mortality rate. HHE can be associated with congestive heart failure, anemia, thrombocytopenia (Kasabach-Merritt syndrome), hepatomegaly and cuta

Prenatal diagnosis has been associated with hydrops fetalis. Postnatal diagnosis is established with US, CT-Scan and MRI. Alpha-fetoprotein levels should be obt

hepatoblastoma. Mortality results from high-output cardiac failure secondary to arteriovenous shunting within the tumor (up to 50% of the cardiac output can be

compromise, hepatic failure, intraperitoneal hemorrhage and consumptive coagulopathy. The younger the age at diagnosis, the more seve re the cardiac symptom

asymptomatic HHE is spontaneous involution. Symptomatic lesions need aggress ive management. Radiotherapy and chemotherapy have not s hown consistently

alpha-interferon are used as initial treatment to inhibit proliferation of endothelial and smooth muscle cells . Symptomatic solitary lesions can be managed with res

disease might nee d hepatic artery e mbolization or transplantation. Hepatic artery ligation or embolization should not be done in patients with shunting from the po

and minimal systemic arterial collateral circulation since it can result in hepatic necrosis.

References:

1- Holcomb GW 3d, O'Neill JA Jr, Mah boub i S, Bishop HC: Experience with h epatic h emangioendot helioma in infancy an d ch ildhoo d. J Ped iatr Surg 23(7):661-6, 1988

2- Becker JM, Heitler MS: Hepat ic hemangioend otheliomas in infancy. Su rg Gynecol Obs tet 168(2):189-200, 1989

3- Gonen R, Fong K, Chiasson DA: Prenatal sonographic diagnosis of hepatic hemangioendothelioma with secondary nonimmune hydrops fetalis. Obstet Gynecol 73(3 Pt 2):485-7, 1989

4- McHugh K, Burrows PE: Infantile hepatic hemangioendotheliomas: significance of portal venous and systemic collateral arterial supply. J Vasc Interv Radiol 3(2):337-44, 1992

5- Davenport M, Hansen L, Heaton ND, Howard ER: Hemangioend othelioma of the liver in infants. J Pediatr Surg 30(1):44-8, 1995

6- Samuel M, Spitz L: Infantile hepatic h emangioendot helioma: the role o f su rgery. J Ped iatr Surg 30(10):1425-9, 1995

7- Daller JA, Bueno J, Gutierrez J, Dvorchik I, Towbin RB, Dickman PS, Mazariegos G, Reyes J: Hepatic hemangioend othelioma: clinical experience and management s trategy . J Ped iatr Surg 34(1):98-105, 1

Candidemia

Candida species (Albicans, Parapsilosis, Tropicalis and Krausei) systemic infection has steadily increased in the neonatal intensive care units during the past yea

type of infection are: prolonged use of broad-spectrum antibiotics, parenteral hyperalimentation, intravenous fat emulsions and placement of a central-venous cat

infections are particularly common when TPN is administered through CVC. Candida can be cultured from the skin, urine, blood and mouth of affected patients. F

abdominal distention are the most common presentations. Infants who are found to have sys temic candidiasis should be treated by removing all factors that predis

(eg., indwelling cathete rs, broad-spectrum antibiotics) as persiste nt fungemia, morbidity and mortality are associated with attempts to maintain the CVC in the pre

initiation of systemic antifungal therapy (amphotericin, fluconazole) is imperative, along with se arching for additional foci of disease. Endophthalmitis, venous thro

complications of CVC associated Candidemia. Once the disease is recognized mortality rates are 20% in infants.

References:

1- Lacey SR, Zaritsky AL, Azizkhan RG: Succes sful treat ment of Candida-infected caval thro mbosis in critically ill infants by low-dos e s treptokinase infus ion. J Pediatr Surg 23(12):1204-9, 1988

7/30/2019 PSU Vol 15, 2000


6/18/12 PSU Vol 15, 2000


2- Leibovitz E, Iuster-Reicher A, Amitai M, Mogilner B: Systemic candidal infections associated with use of peripheral venous catheters in neonates: a 9-year experience. Clin Infect Dis 14(2):485-91, 1992

3- Johns on DE, Thompso n TR, Green TP, Ferrieri P: Systemic cand idiasis in ve ry low-birth-weight infants (less than 1,500 grams). Pediatrics 73(2):138-43, 1984

4- MacDonald L, Baker C, Chenoweth C: Risk factors for cand idemia in a children's h osp ital. Clin Infect Dis 26(3):642-5, 1998

5- Rose HD: Venous cathet er-ass ociated candidemia. Am J Med Sci 275(3):265-9, 1978

6- Stamos JK, Rowley AH: Candidemia in a pediatric population. Clin Infect Dis 20(3):571-5, 1995

7- Eppes SC, Troutman JL, Gutman LT: Outcome of t reatment of candidemia in children whose central cat heters were removed or ret ained. Pediatr Infec t Dis J 8(2):99-104, 1989

8- Dato VM, Dajani AS: Candidemia in children with cent ral venous cathet ers: role of cath eter removal and amphotericin B therapy . Pediatr Infect Dis J 9(5):309-14, 1990

Congenital Lobar Emphysema

Congenital lobar emphysema (CLE) is an unusual lung bud anomaly characterized by massive air trapping in the lung parenchyma that nearly always occurs in inf

commonly (2:1). Lobar over distension causes compression of adjacent lung tissue, mediastinal shift and decrease in venous return. When this occurs persistent p

distress (dyspnea, tachypnea, wheezing, cough and cyanosis) develops requiring lobectomy. Asymptomatic CLE exists, more commonly beyond infancy and assoc

respiratory infection. Lobar hyperinflation, flat diaphragms and retroste rnal air, mediastinal shift in simple films suggests the diagnosis. CT scan depicts the abno

herniation) and the morphology of the remaining lung. V/Q scans confirm the non-functioning nature of the affected lobe. Upper and middle right lobes are more c

centers in a combination of bronchial (flap/valve) obstruction with congenital cartilage dysplasia. Most common associated defect is cardiovascular (VSD, PDA).

always require lobectomy. Asymptomatic children do not benefit from surgical treatment but need close follow-up. Prenatally diagnosed cases need refe rral to sur

References:

1- Murray GF: Congenita l Lobar Emphys ema. Surg Gynec & Obstet. 124: 611-625, 1967 2- Haller JA, Golladay ES, Pickard LR et al: Surgical Management of Lung Bud Anomalies: Lober Emphysema, Bronchogenic Cyst, Cystic Adenomatoid Malformation, and Intralobar Pulmonary Sequestr

1879

3- Markowitz RI, Mercurio MR, Vahjen GA; Gross I, Touloukian RJ: Congenital loba r emphysema. The roles of CT and V/Q scan. Clin Pediatr 28(1):19-23, 1989

4- Nuchtern JG, Harberg FJ: Congenital lung cys ts. Semin Ped iatr Surg 3(4):233-43, 1994

5- Schwartz MZ, Ramachandran P: Congen ital malformations of the lung an d mediastinum--a quarte r centu ry of experience from a s ingle institution . J Pediatr Surg 32(1):44-7, 1997

6- Karnak I, Senocak ME, Ciftci AO, Buyukpamukcu N: Congenital lobar emphys ema: diagnostic an d th erapeutic co nsiderat ions. J Pediatr Surg 34(9):1347-51, 1999

7- Al-Bassam A, Al-Rabeeah A, Al-Nassar S, et al: Congenital cystic disease of the lung in infants and children (experience with 57 cases). Eur J Pediatr Surg 9(6):364-8, 1999

8- Olutoy e OO, Coleman BG, Hubbard AM, A dzick NS: Prenatal diagnos is and management of co ngen ital lobar emphysema. J Ped iatr Surg 35(5):792-5, 2000

Volume 15 No 06 DECEMBER 2000

Abdominal Compartment Syndrome

Elevation of intra-abdominal pressure (IAP) may impair physiology and organ function producing what is known as Abdominal Compartment Syndrome (ACS). Th

of increased intraabdominal pressure consist of cardiac output reduction, pulmonary ventilation res triction (increasing peak inspiratory pressure and hypercapnia

visceral perfusion diminution (gut mucosal acidosis), and increase d in cerebro-spinal pressure. ACS can be the result in abdominal wall defect closures (gastrosch

inflammatory bowel conditions, trauma and intraabdominal infections (enterocolitis, appendicitis, bowel perforation). Vesical and inferior vena cava pressure reco

with IAP. Gastric, rectal, superior vena cava, femoral/brachial artery, and rectus compartment pressure are poor indicators of actual IAP. An elevated abdominal

considered as greater than 25 mm Hg. The bowel is the most sensitive organ to ACS and it develops evidence of end-organ damage before the development of cl

cardiovascular signs. Management consists of abdominal decompress ion. Reopening the abdominal wound is a lifesaving intervention prompted usually by cardio

delayed wound closure (staged celiotomy) may prevent development of this condition in high-risk surgical patients. Timely decompression of the ACS results in i

cardiopulmonary and renal function. Failure to recognize and treat ACS is inevitably fatal.

References:

1-DeCou JM, Ab rams RS, Miller RS, Gauderer MW : Abdo minal compartment s yndrome in children: experience with three cases . J Ped iatr Surg 35(6):840-2, 2000

2- Bendahan J, Coetzee CJ, Papag ianopou los C, Mu ller R: Abdo minal compartment s yndrome. J Trauma 38(1):152-3, 1995 3- Lacey SR, Bruce J, Brooks SP, Griswald J, Ferguson W, Allen JE, Jewett TC Jr, Karp MP, Cooney DR: The relative merits of various methods of indirect measurement of intraabdominal pressure as a g

defects. J Pediatr Surg. 22(12):1207-11, 1987

4- Burch JM, M oore EE, Moore FA, Francios e R: The abdominal compartment s yndrome. Surg Clin North Am 76(4):833-42, 1996

5- Williams M, Simms HH: Abdominal compartment sy ndrome: case reports and implications fo r management in critically ill patients . Am Surg 63(6):555-8, 1997

6- Watso n RA, Howdieshell TR: Abdo minal compartment sy ndrome. South Med J 91(4):326-32, 1998

7- Neville HL, Lally KP, Cox CS Jr: Emergent abdominal decompres sion with patch abdominoplast y in t he p ediatric patient. J Pediatr Su rg 35(5):705-8, 2000

8- Schein M, Wittman DH, Aprahamian CC, et al: The abdominal compartment syndrome: The physiological and clinical consequences of leveated intra-abdominal pressure. J Am Coll Surg 180: 745-753,

Fetal Abdominal Wall Defects

Most common abdominal wall defe cts (AWD) are gas troschisis, omphalocele and hernia of the umbilical cord. Refe rral to te rtiary centers with available neonatal

in prenatally diagnosed cases. Changing the route of delivery does not affect outcome for either defect. Omphalocele has a high incidence of associated anomalie

genitourinary, skeletal, chromosomal syndromes) that are the cornerstones of mortality. Detailed search for associated anomalies, fetal echocardiogram and kar

always. Cesarean section is justified in large omphaloceles (> 5 cm) to avoid liver damage, sac rupture and dystocia. Gastroschisis prenatal US appearance depe

condition of extruded bowel. Fetal karyotyping testing is less important. Intestinal atresia complicates the defect, the result of an intrauterine vascular accident. I

atresia or luminal constriction may cause polyhydramnios, fetal growth retardation and preterm labor, findings that can be monitored with serial US. No benefit h

recommending routine c-section for most cases of gastroschisis. Preterm deliveries by c-section have been found to prevent bowel damage in fetus with progressi

thickening, a finding that has not been corroborated by others. Abnormal US appearance of fetal bowel is as sociated with more bowel edema, longer operative ti

postoperative complications.

References: 1- Langer JC: Fetal Abdominal Wall Defects. Semin Pediatr Surg 2(2):121-128, 1996

2- Dykes EH: Prenatal diagno sis and management of abdominal wall defects. Semin Pediatr Surg. 5(2):90-4, 1996

3- Sipes SL, Weiner CP, Sipes DR 2d, Grant SS, Williamson RA: Gastroschisis and omphalocele: does either antenatal diagnosis or route of delivery make a difference in perinatal outcome? Obstet Gynec

4- Lewis DF, Towers CV, Garite TJ, Jackson DN, Nageotte MP; Major CA: Fetal gastroschisis and omphalocele: is cesarean section the best mode of delivery? Am J Obstet Gynecol 163(3):773-5, 1990

5- Lurie S, Sh erman D, Bukovsky I: Omphalocele delivery enigma: the bes t mode of delivery s till remains d ubious . Eur J Obs tet Gyneco l Reprod Biol 82(1):19-22, 1999

6- Raynor BD, Richards D: Growth retardation in fetuses with gastroschisis. J Ultrasound Med 16(1):13-6, 1997

7/30/2019 PSU Vol 15, 2000


6/18/12 PSU Vol 15, 2000


7- Bethel CA, Seashore JH, Touloukian RJ: Cesarean section does not improve outcome in gastroschisis. J Pediatr Surg 24(1):1-3, 1989

8- Langer JC, Khanna J, Caco C, Dykes EH, Nicolaides KH: Prenatal diagnosis of gastroschisis: development of objective sonographic criteria for predicting outcome. Obstet Gynecol 81(1):53-6, 1993

9- Lenke RR, Persutte WH, Nemes J: Ultrasonographic assessment of intestinal damage in fetuses with gastroschisis: is it of clinical value? Am J Obstet Gynecol 163(3):995-8, 1990

10- Alsulyman OM, Monteiro H, Ouzounian JG, Barton L, Songster GS; Kovacs BW: Clinical significance of prenatal ultrasonographic intestinal dilatation in fetuses with gastroschisis. Am J Obstet Gyn

11- Adra AM, Landy HJ, Nahmias J, Gomez-Marin O: The fetus with gastroschisis: impact of route of delivery and prenatal ultrasonography. Am J Obstet Gynecol 174(2):540-6, 1996

Cholecystokinin

Cholecystokinin (CCK) is a naturally occurring octapeptide hormone that has several utilities in children. Secreted in the proximal small bowel, increases bile flo

gallbladder and promotes GI and colonic motility. As diagnostic source is used in determining the ejection fraction of the biliary tree . Therapeutically, CCK has b

in cases of TPN cholestasis. CCK is associated with functional and histologic improvement in the periportal area of the liver as well as preservation of gallbladde

significantly diminishes direct bilirubin levels in infants with TPN cholestasis, effectively clears the biliary tree from sludge and stones. CCK use can be associate

pain, feeding intolerance, flushing and rarely hypotension. Children with clinical liver failure have no response to CCK. If conjugated hyperbilirubinemia from TP

weeks of full enteral feedings and stools remain acholic, CCK therapy should be considered. Evidence-based data that CCK prevents TPN cholestasis is not conc

resuming enteral feeding is the only effective management in TPN cholestasis.

References:

1- Lugo-Vicente HL: Gallbladder Dyskinesia in Children. Jo urnal of Society o f Laparoend oscopic Surgeons 1(1):61-65, 1997

2- Curran TJ, Uzoaru I, Das JB, Ansari G, Raffensperger JG: The effect of cholecystokinin-octapeptide on the hepatobiliary dysfunction caused by total parenteral nutrition. J Pediatr Surg 30(2): 242-247,

3- Teitelbaum DH, Han-Markey T, Sch umacher RE: Treatment of p arenteral nu trition-associated cholest asis with cholecys tokinin-octapept ide. J Pediatr Surg 30(7): 1082-1085, 1995

4- Moss L, Amii LA: New Approaches to understanding the etiology and treatment of total parenteral nutrition-associated cholestasis. Sem Pediatr Surg 8(3): 140-147, 1999

5- Rintala RJ, Lindahl H, Pohjavuori M: Total parenteral nutrition-associated cholestasis in surgical neonates may be reversed by intravenous cholecystokinin: A preliminary report. J Pediatr Surg 30(6):

Home Table Index Past Review Submit Techniques

Handbook Articles Download UPH Journal Club WWW Meetings

Documents

PSU Vol 15, 2000