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PEDIATRIC SURGERY UPDATE

VOLUME 19, 2002

Volume 19 No 01 JULY 2002

Laparoscopic Inguinal Herniography

Besides using the laparoscopic te chnique for identifying a contralateral patent processus vaginalis using a trans-inguinal approach or repair a recurrent inguinal h

approach can also be use for repair of inguinal hernias in children. The technique consists in closing the neck of the hernial sac with a purse string suture. Advant

include the ability to diagnose and repair concomitantly a contralateral hernia defect due to excellent visual effect, the ability to diagnose a direct inguinal hernia,

less surgical dissection. This last issue is called "access trauma" or repair of all the damage caused in gaining the initial access to ligate a hernial sac. Another be

dissection of the cord structures so that the risk of superior displacement of the testis, testicular atrophy, injury to vas deferens and stitch granuloma is less likel

longer procedure, higher recurrent rates, development of residual hydrocele in males along with the complications attendant in violating the abdominal cavity (vas

caused by Veress needle and trocar insertion). Most surgeons still prefer to do an open subcutaneous procedure with high ligation of hernia sacs as standard rep

infants and children.

References:

1- Schier F, Montu pet P, Esp os ito C: Laparos copic Ing uinal Herniography in Children: A Three-Center Experience with 933 repairs. J Pediatr Surg 37(3): 395-397, 2002

2- Geisler DP, Jegathes an S, Parmley MC, McGee JM, Nolen MG, Broughan TA : Laparoscop ic exploration for t he clinically un detect ed he rnia in infancy an d childhoo d. Am J Surg 182(6):693-6, 2001

3- Schafer M, Lauper M, Krahenbuhl L: Trocar and Veress needle injuries during laparoscopy.Surg Endosc 15(3):275-80, 2001

4- Schier F: Direct inguinal he rnias in ch ildren: laparos copic as pects . Pediatr Surg In t 16(8):562-4, 20005- Schier F: Laparosco pic su rgery o f inguinal hern ias in children--initial experience. J Pediatr Surg 35(9):1331-5, 2000

6- Montu pet P, Esp osito C: Laparos copic Treat ment of Congenital Inguinal Hernia in Children. J Ped iatr Surg 34(3): 420-423, 1999

Congenital Diaphragmatic Hernia Update

Congenital Diaphragmatic Hernia (CDH) continues to carry a high mortality and morbidity. The mortality is the result of lethal pulmonary hypoplasia, the presenc

anomaly (usually a cardiac defect), ce ntral nervous system bleeding/injury from aggressive medical management (i.e ., ECMO), or iatrogenic barotrauma. Lethal

probably res t its solution in fetal intervention as more prenatal techniques are use d to identify the fe tus with such a problem (progress ive ipsilateral pulmonary ar

fetal e cho-doppler and fetal lung volume measurement by magnetic resonance imaging correlates with progressive fetal lung hypoplasia and worse postnatal outc

trends in management of babies suffering from CDH res t on three principles that have bee n found to reduce mortality and morbidity: 1) use of soft mechanical ve

mechanical ventilation and minimal inspiratory pressure settings) with permissive hypercapnia, 2) delay surgical repair until hemodynamic, ventilatory and acid-b

ipsilateral chest tube placement s ince they cause negative pressure gradient reducing lung deflation leading to lung injury and barotrauma.

References:

1- Boloker J, Bateman DA, Wung JT, Stolar CJ: Congenital Diaphragmatic Hernia in 120 Infants Treated Consecutively with Permissive Hypercapnia/Spontaneous Respiration/Elective Repair. J Pediatr S

2- Sokol J, Bohn D, Lacro RV, Ryan G, Stephens D, Rabinovitch M, Smallhorn J, Hornberger LK: Fetal pulmonary artery diameters and their association with lung hypoplasia and postnatal outcome in co

Obstet Gynecol 186(5 Pt 1):1085-1090, 20023- Mahieu-Caputo D, Sonigo P, Dommergues M, Fournet JC, Thalabard JC, Abarca C, Benachi A, Brunelle F, Dumez Y: Fetal lung volume measurement by magnetic resonance imaging in congenital diap

2001

4- Kays DW, Lang ham MR, Ledbetter DJ, et al: Detrimental Effects of Stand ard Med ical Therapy in Cong enital Diaphragmatic Hernia. Ann Surg 230: 340-351, 1999

5- Serrano P, Reyes G, Lugo-Vicente HL: Congen ital Diaphrag matic Hernia: Mortality Determinants in a Hispanic Popu lation. PR Health Science J 17(4): 317-321, 1998

Fecal Incontinence

Fecal incontinence (FI) in children is usually the result of a congenital (imperforate anus, Hirschsprung's disease and myelomeningocele) or acquired (trauma, pel

spinal cord injury) condition. FI causes a psychological, developmental and social stress of great magnitude. Assessment of the problems includes the use of anal

and pudendal nerve te rminal motor latencies. Rectal emptying and angulation can be evaluated with defe cograms, MRI or scintigraphic proctography. The manaHD biopsychosocial treatment consisting of e xplanation, extinction of fear and avoidance behavior, learning new defecation behavior, learning an adequate strain

generalization toward daily life have been effective. Biofeedback is a harmless and inexpensive treatment coordinating pelvic floor muscle contraction with the se

has brought partial success in children with FI. Other less effective forms of treatment are dynamic graciloplasty and sacral nerve stimulation. Functional constip

incontinence and the presence of se gmental dilatation of the sigmoid or rectum responds poorly to medical management. The megabowel lacks an adequate anore

propulsive function causing a functional obstruction. Segmental resection of the dilated segment has brought successful relief of symptoms. Antegrade enemas by

appendicocecostomy or cecostomy tube can achieve total colonic evacuation and socially controlled continence in children with anorectal malformations and caud

percentage of cases.

References:

1- Darakhsan A A, Williams NS: Recent Inno vations in the M anagement of Fecal Incon tinence. Sem Pediatr Surg 11(2): 83-90, 2002

2- van Kuyk EM, Brugman-Boezeman AT, Wissink-Essink M, Severijnen RS, Festen C, Bleijenberg G: Defecation problems in children with Hirschsprung's disease: a biopsychosocial approach. Pediatr

3- Heymen S, Jones KR, Ringel Y, Scarlett Y, Whitehead WE: Biofeedback treatment o f fecal incont inence: a critical review. Dis Colon Rectum 44(5):728-36, 2001

4- Lee SL, DuBois JJ, Montes-Garces RG, Inglis K, Biediger W: Surgical management of chronic unremitting constipation and fecal incontinence associated with megarectum: A preliminary report. J Pedi

5- Whitehead WE, Wald A, Norton NJ: Treatment op tions for fecal incontinence . Dis Colon Rectum 44(1):131-42, 20016- Lee SL, Rowell S, Greenholz SK: Therapeutic cecos tomy tubes in infants with imperforate an us and caudal agen esis. J Pediatr Surg 37(3):345-7, 2002

7- Rintala RJ: Fecal incont inence in ano rectal malformations, neu ropathy , and miscellaneous conditions . Semin Pediatr Surg 11(2):75-82, 2002

Volume 19 No 02 AUGUST 2002

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Ovarian Hernia

Inguinal hernias are the most common surgical pathology seen in infants and children. A lump in the inguinal canal area of a newborn or infant female is most prob

inguinal hernia. On very rare occas ion the lump is a testis in a child with testicular feminization syndrome. Clinically the irreducible ovarian lump is usually asymp

tender mass within the labia majora. Ultrasound can determine the nature of the gonad present. The main problem with an ovarian hernia in infants is the incidenc

associated before repair. Ovaries trapped within inguinal hernias undergo torsion far more commonly than ovaries and tubes in the normal pelvic position increas

Torsion can occur at any time after diagnosis of the hernia. The incarcerated ovarian pedicle is narrowed and lengthened within the defect and the internal ring se

which a twist can occur. The risk of torsion and infarction creates the view that ovarian hernias s hould be repaired at the earliest elective opportunity if they can b

Children with edema, tenderness or skin discoloration in the inguinal area should be repaired immediately. Early recognition and management of this condition re

infarction. During repair surgeons must be aware that in 20% of girls with inguinal hernia, the fallopian tubes occasionally with the ovary or uterus comprise the

component).

References:

1- Goske MJ, Emmens RW, Rabinowitz R: Inguinal ov aries in ch ildren d emonstrat ed by high res olution real-time ultrasoun d. Radiology 151(3):635-6, 1984

2- Boley SJ, Cahn D, Lauer T, Weinberg G, Kleinhaus S: The irreducible ovary: a true emergency . J Pediatr Surg 26(9):1035-8, 1991

3- Ozbey H, Ratsch ek M, Schimpl G, Hollwarth ME: Ovary in hernia sac: prolaps ed or a d escended gonad ? J Pediatr Surg 34(6):977-80, 1999

4- Merriman TE, Auldist AW: Ovarian to rsion in ingu inal hernias. Ped iatr Surg Int 16(5-6):383-5, 2000

Diversion Colitis

Diversion colitis is an inflammatory state resulting from a nutritional deficiency in the lumen of the colonic epithelium. The colitis develops in segments of the colo

diversion of the fecal stream persisting indefinitely unless the excluded segment is reanastomosed. Diversion colitis is characterized by rectal discomfort, pain, di

bleeding. Symptoms occur three months or more after bowel diversion. Diagnosis is e stablished by colonic or rectal biopsy. Histologic abnormalities included apht

atrophy and abscesses , a villous colonic surface, and a mixed acute and chronic inflammatory infiltrate with patchy lymphoid hyperplasia. This condition is caused

short-chain fatty acids, the preferred metabolic substrates of colonic epithelium. In children diversion colitis can be seen in bowel derived Hirschsprung's disease,colitis and Crohn's disease. It has also been reported to also occurs after sigmoid neovagina reconstruction. Adjunctive management includes the use of a topical

acids (propionate, acetate, butyrate) and/or 5-Aminosalicylic acid (5-ASA) to control symptoms. Definitive treatment consists of excision of rectum or stomal clos

References:

1- Harig JM, So ergel KH, Komorowski RA, Woo d CM: Treatment of d iversion co litis with s hort-cha in-fatty acid irrigation. N Engl J Med 320(1):23-8, 1989

2- Komorowski RA: Histologic s pectrum of diversion colitis. Am J Surg Pathol 14(6):548-54, 1990

3- Toolenaar TA, Freundt I, Huikeshoven FJ, Drogendijk AC, Jeekel H, Chadha-Ajwani S: The occurrence of diversion colitis in patients with a sigmoid neovagina. Hum Pathol 24(8):846-9, 1993

4- Grant NJ, Van Kruiningen HJ, Haque S, Wes t AB: Mucos al inflammation in ped iatric diversion colitis: a quant itative analys is. J Pediatr Gastroent erol Nutr 25(3):273-80, 1997

5- Kiely EM, Ajayi NA, Whee ler RA, Malone M: Diversion Procto-Colitis: Respons e to Treatment with Short -Chain Fatty A cids. J Pediatr Surg 36(10): 1514-1517, 2001

Duodenal Atresia

Duodenal atresia (DA) is the most common congenital anomaly associated with the duodenum and the most common atresia found in the GI tract. The double-bubstomach and duodenal bulb seen prenatally (US) as two anechoic cysts or after birth (simple abdominal films) is characteristically diagnostic. DA is associated one

Down's syndrome followed by cardiac malformations (20%). Prenatal karyotyping and fetal echocardiogram of suspected DA case s will es tablish the association.

presents with bilious vomiting and epigastric distension (dilated stomach) depending whether the atres ia occurs proximally (10%) or distally (90%) to the papilla o

from DA needs urgent differentiation from malrotation which carries the risk of midgut volvulus. If in doubt a small upper GI series using a water soluble contrast

correction of ele ctrolytes imbalances, management of DA consis ts of diamond-shaped duodeno-duodenostomy. If the proximal duodenum is massive ly dilated a ta

(imbrication or stapler resection) will help reduce the poss ibilities of a functional anastomotic obstruction. Late complications include motility disorders, megaduo

reflux, duodenal-gastric reflux, gas tritis, peptic ulcer disease , blind loop syndrome and biliary-pancreatic conditions which may be observed months to years after

References:

1- Ein SH, Kim PC, Miller HA: The late nonfu nctioning duod enal atres ia repair--a second look. J Pediatr Surg 35(5):690-1, 2000

2- Murshed R, Nicholls G, Spitz L: Intrinsic duodenal obstruction: trends in management and outcome over 45 years (1951-1995) with relevance to prenatal counseling. Br J Obstet Gynaecol 106(11):1197-

3- Takahashi A, Tomomasa T, Suzuki N, Kuroiwa M, Ikeda H, Morikawa A, Matsuyama S, Tsuchida Y: The relationship between disturbed transit and dilated bowel, and manometric findings of dilated b

atres ia and stenos is. J Ped iatr Surg 32(8):1157-60, 1997

4- Upadhyay V, Sakalkale R, Parashar K, Mitra SK, Buick RG, Gornall P, Corkery JJ: Duod enal atres ia: a comparison of th ree modes of treat ment. Eur J Pediatr Surg 6(2):75-7, 1996

5- Grosfeld JL, Rescorla FJ: Duodenal atresia and stenosis: reassessment of treatment and outcome based on antenatal diagnosis, pathologic variance, and long-term follow-up. World J Surg 17(3):301-9,

6- Akhtar J, Guiney EJ: Congen ital duodenal obs truction. Br J Surg 79(2):133-5, 1992

7- Kimura K, Mukohara N, Nishijima E, Muraji T, Tsu gawa C, Mats umoto Y: Diamond-sh aped anas tomosis for duo denal atres ia: an experience with 44 patient s o ver 15 years . J Pediatr Surg 25(9):977-9, 19

Volume 19 No 03 SEPTEMBER 2002

Parotid Mass

A parotid mass in a child creates great concern and should be managed promptly since a high percentage of cases will harbor a malignant tumor. The more comm

children includes hemangioma, pleomorphic adenoma and lymphangiomas. Infants with a rapidly enlarging violaceous or soft tumor in the parotid region harbors

Hemangioendothelioma is the most common parotid gland tumor of childhood. They seldom need e xcision as spontaneous regression is the norm. An asymptomat

the most common presentation in older children. Tenderness is associated with an infectious process. Diagnosis includes the use of Doppler ultrasound, CT-Scan,

aspiration (FNA) biopsy. Salivary gland carcinoma is rare in children. Most common histological type is mucoepidermoid either as a primary neoplasm or se condirradiation. Management consists of superficial or total parotidectomy extent which is se lected during the surgical procedure depending on deep gland or fascial n

must be widely excised. Re currence is managed with postoperative irradiation for high or intermediate grade malignancies. Rhabdomyosarcoma arising in the par

locally invasive aggressive tumor that presents early with swelling and symptoms of seventh nerve deficit. This tumor needs surgical excision followed by chemot

References:

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1- Zurrida S, Alasio L, Tradati N, Bartoli C, Chiesa F, Pilotti S: Fine-needle as piration of pa rotid masses. Cancer 72(8):2306-11, 1993

2- Rogers DA, Rao BN, Bowman L, Marina N, Fleming ID, Schrop p KP, Lobe TE: Primary malignancy of the salivary g land in ch ildren. J Pediatr Surg 29(1):44-7, 1994

3- Khadaroo RG, Walton JM , Ramsay JA, Hicks MJ, Archibald SD: Mucoep idermoid carcinoma of the parotid gland : a rare presen tation in a y oung child. J Pediatr Surg 33(6):893-5, 1998

4- Salomao DR, Sigman JD, Greenebaum E, Cohen MB: Rhabdomyos arcoma presen ting as a parotid g land mass in pediatric patients : fine-need le aspiration b iopsy findings. Cancer 84(4):245-51, 1998

5- Roebuck DJ, Ahuja AT: Hemangioendoth elioma of the parotid gland in infants: sonog raphy an d correlative MR imaging. AJNR Am J Neuroradiol 21(1):219-23, 2000

6- Orvidas LJ, Kasp erbauer JL, Lewis JE, Olsen KD, Lesn ick TG: Pediatric parotid masses . Arch Otolaryngol Head Neck Surg 126(2):177-84, 2000

Perineal Hemangioma

Hemangiomas continue to be the most common benign tumor in infants. Perineal hemangiomas involving the scrotum, perianal region and rectum have been spor

the anogenital area have a strong predilection for females . The clinical prese ntation can start with an undiagnosed non-healing ulcer, a pale macule, erythematous

stain in the perineal region. Recurrent perianal blood loss can be caused by a rectal hemangioma. Local factors such as abrasion or maceration plays a potentiati

MRI helps determine the extension of the hemangioma toward the pelvis and surrounding tissues. Though most hemangiomas spontaneously involute over a peri

the perineal and ano-rectal region are troublesome lesions causing repeated ulcerations and subsequent pain. The subcutaneous perineal hemangioma can be ma

sys temic ste roids or using alpha interferon therapy depending on the symptomatology of the child. Diffuse cavernous hemangioma of the colon, rectum and anus c

temporary colostomy followed by modified endorectal pull-through.

References:

1- Liang MG, Fireden IJ: Perineal and Lip Ulceration as the Pres enting M anifestat ion of h emangioma of Infancy. Ped iatrics 99(2): 256-259, 1997

2- Bouchard S, Yazbeck S, Lallier M: Perineal hemangioma, ano rectal malformation, an d genital anomaly: a new a ss ociation? J Pediatr Surg 34(7):1133-5, 1999

3- Pohlen U, Kroesen AJ, Berger G, Buhr HJ: Diagnostics and surgical treatment strategy for rectal cavernous hemangiomas based on three case examples. Int J Colorectal Dis 14(6):300-3, 1999

4- Takamatsu H, Akiyama H, Noguchi H, Tahara H, Kajiya H: Endorectal pull-through operation for diffuse cavernous hemangiomatosis of the sigmoid colon, rectum and anus. Eur J Pediatr Surg 2(4):245

5- Achau er BM, Vander Kam VM: Ulcerated anogen ital hemangioma of infancy. Plast Recon str Surg 87(5):861-6, 1991

Fetus-in-Fetu

Fetus-in-fetu is a ve ry rare condition in which a malformed parasitic twin is found inside the body of its partner as an abdominal fetiform calcified mass. They are

retroperitoneum. Symptoms relate to the mass e ffect and include abdominal distension, feeding difficulty, vomiting and dyspnea. It represents an abortive attemp

one fetus (the parasite) is drawn into the abdominal cavity of the host fetus (the autosite) in early intrauterine life and is attached retroperitoneally, with the blood

supplied by the host superior mesenteric vessels . There exist much controversy in the literature whether a fetus-in-fetu is a well-formed teratoma (tumor compos

layers) or not. To be coined fetus-in-fetu the mass must demonstrate true organogenesis and an axial skeleton separating them from teratomas, which never unde

Nonvisualization of the vertebral axis on radiography or on CT Scan does not exclude the diagnosis of fetus-in-fetu as it can be seen by the pathologist. Chromoso

shows normal chromosomes identical to the host. Management consists of complete surgical resection in as much as malignant recurrence has been reported.

References:

1- Eng HL, Chuang JH, Lee TY, Chen W J: Fetus in fetu: a cas e report and review of the literature. J Pediatr Surg 24(3):296-9, 1989

2- Hopkins KL, Dickson PK, Ball TI, Ricketts RR, O'Shea PA, A bramowsky CR: Fetus-in-fetu with malignan t recurren ce. J Pediatr Surg 32(10):1476-9, 1997

3- Hoeffel CC, Nguyen KQ, Phan HT, Truong NH, Nguyen TS, Tran TT, Forn es P: Fetus in fetu: a case report an d literature review. Pediatrics 105(6):1335-44, 2002

4- Mills P, Bornick PW, Morales WJ, A llen M, Gilbert-Barness E, Johns on PK, Quintero R: Ultraso und p renatal diagno sis o f fetus in fetu. Ultrasoun d Obst et Gynecol 18(1):69-71, 20015- Federici S, Prestipino M, Domenichelli V, Ant onellini C, Sciutti R, Domini R: Fetus in fetu: repo rt of an ad ditional, well-developed case. Pediatr Su rg Int 17(5-6):483-5, 2001

Volume 19 No 04 OCTOBER 2002

Colonic Atresia

Colonic Atresia (complete absence of the lumen of the colon) is a rare cause of congenital bowel obstruction comprising almost 5% of all cases of bowel atresia in

(CA) can be associated with distal skeletal deformities, imperforate anus, Hirschsprung's and abdominal wall defects. Complicated cases are associated with part

and severe deformities of the genitourinary tract (bladder and cloacal exstrophy). Colonic Atresia is the result of an intrauterine mesenteric vascular accident. Cl

distension, bilious vomiting and obstipation. Simple abdominal films shows hugely distended distal bowel loops and air-fluid levels. Barium enema will show a micr

intervention is urgent since a competent ileo-cecal valve makes the obstruction a close loop. During surgery patency of the gastrointestinal tract must be confirm

avoid missing a second associated atresia. For atresias located in the right colon, resection and end to oblique primary anastomosis is recommended. Atresias loc

critically ill children or complex combinations of abse nt hindgut should be initially managed with a diverting colostomy. Suspect the coexistence of Hirschsprung'swhen a functional obstruction is present after repair of the atresia. Suction rectal biopsy before closing the colostomy should solve this problem. Prognosis depen

associated malformations.

References:

1- Coran A G, Eraklis AJ: At resia o f the colon. Su rgery 65: 828-831, 1969

2- Powell RW, Raffensperger JG: Congenital Colonic Atresia. J Pediatr Surg 17(2): 166-170, 1982

3- Pohlso n EC, Hatch EI Jr, Glick PL, Tapper D: Indiv idualized manag ement of co lonic atre sia. Am J Surg 155(5):690-2, 1988

4- Davenport M, Bianchi A, Doig CM, Gough DC: Colonic atresia: current res ults o f treatment. J R Coll Surg Edinb 35(1):25-8, 1990

5- Williams MD, Burringto n JD: Hirschs prung's diseas e complicating colon atres ia. J Pediatr Surg 28(4):637-9, 1993

6- Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ, Sch erer LR, Engum SA: Intes tinal atres ia and s tenos is: a 25-year experience with 277 case s. A rch Surg 133(5):490-6, 1998

7- Akgur FM, Olguner M, Hakguder G, Ozer E, Aktug T: Colonic atresia ass ociated with Hirschs prung's diseas e: it is n ot a d iagnos tic challenge. Eur J Pediatr Surg 8(6):378-9, 1998

8- Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Buyukpamukcu N: Colonic atres ia: surgical management and outco me. Pediatr Surg Int 17(8):631-5, 2001

Colonic Strictures

Colonic strictures in infants are most commonly the result of a late complication from neonatal necrotizing enterocolitis (NEC). Other times they can be congenita

children receiving high dose pancreatic enzyme supplement (cystic fibrosis) or after inflammatory bowel diseas e. Post-NEC strictures occur both after medical an

medically-treate d infants symptoms of bowel obstruction usually begin six to eight wee ks after resolution of NEC, while s urgically managed infants deve lop asy

an enterostomy. The clinical manifes tations of colonic strictures include abdominal distention, bilious vomiting, hematochezia, diarrhea, disaccharide intolerance,

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loss and growth failure. Splenic flexure and left colon are the most common sites of NEC stricture formation. Contrast study of the distal bowel can establish the

case s. Although routine studies have no advantage over clinical follow-up, some studies propose a diagnostic protocol using an early upper GI s tudy with non-ioni

material followed by a contrast enema in suspicious cases as a reliable method of diagnosing strictures. Contrast enemas are essential prior to restoring bowel co

Balloon catheter dilatation of the stricture has been tried but recurrence closure rates are too high to be recommended as definite therapy. Once identified manag

and primary anastomosis of the stricture.

References:

1- Kosloske AM, Burste in J, Bartow SA: Intest inal obstruc tion due to colonic stricture following neona tal necrotizing enterocolitis. Ann Su rg 192(2):202-7, 1980

2- Ball WS Jr, Seigel RS, Goldthorn JF, Kosloske AM: Colonic st rictures in infan ts following inte stinal isch emia. Treatment b y balloon cathet er dilatation. Radiology 149(2):469-71, 1983

3- Hartman GE, Drugas GT, Shochat SJ: Post-necro tizing ent erocolitis st rictures pres enting with seps is or perforat ion: risk of clinical obse rvation. J Pediatr Surg 23(6):562-6, 1988

4- Nanjundiah P, Lifschitz CH, Gopalakrishna GS, Cochran WJ, Klish WJ: Intes tinal strictures presen ting with gas trointes tinal blood loss . J Pediatr Surg 24(2):174-6, 19895- Radhakrishnan J, Blechman G, Shrader C, Patel MK, Mangurten HH, McFadden JC: Colonic strictures following successful medical management of necrotizing enterocolitis: a prospective study evalu

st udies . J Pediat r Surg 26(9):1043-6, 1991

6- Collins MH, Azzarelli B, West KW, Chong SK, Maguiness KM, Stevens JC: Neuropath y and vascu lopathy in colon ic strictures from children with cystic fibrosis . J Pediatr Surg 31(7):945-50, 1996

Colonic Volvulus

Volvulus of the colon represent a rare cause of bowel obstruction in children. Colonic volvulus can occur in the sigmoid (most common site), transverse and cecal

same pathogenetic mechanism: a long freely redundant mobile colon and mesocolon, lack of fixation and short mese nteric attachment of the proximal and distal

volvulus have been associated to chronic constipation, high fiber diet, mental retardation and Hirschsprung's dise ase . Central abdominal pain of sudden onse t foll

distension, tenderness , bloody mucoid discharge or inability to pass flatus is characteristic. Simple abdominal films will show large bowel dilated loops with air-flui

showing a bird beak appearance of the colon and air-contrast mirror image in the proximal end is diagnostic, and in a few cases therapeutic. Management is oper

enema reduction is associated with an unacceptable high rate of recurrence. Surgery consist of resection of the involved part with end-to-end anastomosis for limi

colopexy for extensive bowel involvement.

References:

1- Mellor MF, Drake DG: Colonic volvulus in children: value of barium enema for diagn osis and t reatment in 14 children. AJR Am J Roentg enol 162(5):1157-9, 1994

2- Ismail A: Recurrent colonic volvulus in children. J Pediatr Surg 32(12):1739-42, 1997

3- Samuel M, Boddy SA, Nicholls E, Capps S: Large bowel volvulus in childhood. Aus t N Z J Su rg 70(4):258-62, 2000

4- Mercado-Deane MG, Burton EM, Howell CG: Transv erse colon vo lvulus in p ediatric patients . Pediatr Radiol 25(2):111-2, 1995

5- Houshian S, Soren sen JS, Jensen KE: Volvulus of the t ransv erse colon in children. J Pediatr Surg 33(9):1399-401, 1998

6- Salas S, An gel CA, Salas N, Mu rillo C, Swisch uk L: Sigmoid volvulus in children and adolescents . J Am Coll Surg 190(6):717-23, 20007- Samuel M, Boddy SA , Capps S: Volvulus of th e trans verse and s igmoid colon. Ped iatr Surg Int 16(7):522-4, 2000

Volume 19 No 05 NOVEMBER 2002

Esophago-Gastric Dissociation

Gastroesophageal reflux is a common and distress ing problem in neurologically affected children. Couple with diminished salivary clearance, pharyngo-esophage

esophageal dysmotility the incidence of pulmonary aspiration and infection is increased in this pediatric population. Fundoplication and gastrostomy are effective

reducing aspiration and improving quality of care. Unfortunately, a large number of these kids develop complications after the fundoplication. They include: wrap

or paraesophageal), disruption, gagging, retching and recurrent preop GER symptoms (vomiting and pneumonia are the most troublesome). Theories explaining t

supine position, swallowing incoordination, esophageal dysmotility, spasticity, seizures, delayed gastric emptying, chronic constipation and scoliosis (increase intr

Recurrent reflux can be managed with redo fundoplication, though postoperative complications increase significantly. A viable alternative is e sophago-gastric diss

consists of closing the e sophagogastric junction and establishing bowel continuity with a Roux-en-y es ophagojejunostomy and an end-to-side jejuno-jejunostomy w

the gastrostomy along with unrestricted oral intake. The procedure allows free passage of saliva avoiding the obstructive nature of a fundoplication (tight wrap) i

dysmotility. EGD is indicated in children with pathological reflux and seve rely neurological disorders, poor or none oral feeding and difficult to manage pharyngo-

and esophageal dysmotility.

References:1- Pearl RH, Robie DK, Ein SH, Shandling B, Wes son DE, Superina R, Mct aggart K, Garcia VF, O'Conno r, Filler RM: Complications o f gas troes ophag eal antireflux surgery in neurologically impaired vers u

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

2- Bianchi A: To tal Esoph agogastric Dissociation: An Alternative A pproach. J Pediatr Su rg 32(9): 1291-1294, 19973- Danielson PD, Emmens RW: Esophag ogas tric Disconn ection fo r Gastroesophageal Reflux in Children with Seve re Neurological Impairment. J Pediatr Surg 34(1): 84-87, 19994- Gatti C, Federici di Abriola G, Villa M, De Angelis P, Laviani R, La Sala E, Dall'Oglio L: Esophagogas tric Dissociation versu s Fu ndop lication: Which is Bes t for Seve rely Neurologically Impaired Childre

Testicular Torsion

Whenever an infant or child develops acute scrotal pain, swelling and/or redness of the scrotum the diagnosis of testicular torsion should be highly entertained. Te

most common pediatric urological emergency. TT can occur extravaginally or intravaginally. Extravaginal TT is a perinatal event (in utero) which occurs when the

torsion at the level of the external ring causing strangulation of all the scrotal content. The infant present with a firm, hard, painless scrotal mass. In few cases ex

tes tis. The infarcted testis is removed and the contralateral testis pexed to avoid a future problem of this nature. Intravaginal TT occurs due to an anomalous att

vaginalis (bell clapper deformity) causing the gonad to twist upon its vascular pedicle. Due to the disparity in size and weight of pubertal testis, torsion occurs mos

Child develops sudden scrotal pain, edema, nausea and vomiting. Cremasteric reflex is lost. Color Doppler sonography (readily available and non-invasive) in exp

Technetium scan are both reliable in suggesting the diagnosis. Immediate scrotal exploration is the best means available of establishing the diagnosis and saving

remove a dead testis or relieve the torsion the contralateral testis should also be pexed.

References:

1- Hermann D: The ped iatric acute scrot um. Pediatr Ann 18(3):198-202, 1989

2- Cilento BG, Najjar SS, At ala A: Cryptorch idism and te sticular t orsion. Pediatr Clin North Am 40(6):1133-49, 1993

3- Kass EJ, Lundak B: The acute s crotum. Pediatr Clin North Am 44(5):1251-66, 1997

4- Duncan ND, Gabay L, Coard KC, Antoine M, McDonald A: Torsion of an intra-abdominal testicle in a neonate. Case report and review of the literature. West Indian Med J 46(4):126-7, 1997

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5- Burgher SW: Acute s crotal pain. Emerg Med Clin North Am 16(4):781-809, 1998

6- Munden MM, Trau twein LM: Scrotal pathology in pediatrics with son ograph ic imaging. Curr Probl Diagn Radiol 29(6):185-205, 2000

Alkaline Reflux

Alkaline (pH > 7) gas troesophageal reflux (AGER) is not so rare in children affe cting 10% of all refluxers. Acid reflux is more common (50%), followed by combi

(20%). AGER causes gastritis and esophagitis and is associated with repetitive vomiting, excessive crying and chronic respiratory disease. In an alkaline enviro

deconjugated bile salts produce the greatest injury to the esophageal mucosa. Simultaneous gastroesophageal pH monitoring can help establish the diagnosis. Me

aimed to the individual pH pattern. Use of prokinetics to enhance motility might be a better solution in the child with AGER. The child with an antireflux procedur

dysfunction might continue with dyspeptic symptoms and gastritis needing further therapy. The aim is guide clinicians to choose efficient anti-reflux therapy base

References:

1- Vanden plas Y, Loeb H: Alkaline gas troesophag eal reflux in infancy. J Ped iatr Gast roenterol Nutr 12(4):448-52, 1991

2- Malthaner RA, Newman KD, Parry R, Duffy LF, Randolph JG: Alkaline gas troesophageal reflux in infants and ch ildren. J Pediatr Surg 26(8):986-90, 1991

3- Tovar JA, Wang W , Eizaguirre I: Simultaneou s gastro eso phageal pH monitoring an d the diagnos is of alkaline reflux. J Pediatr Surg 28(10):1386-91, 1993

4-Wenzl TG, Silny J, Schenke S, Peschgens T, Heimann G, Skopnik H: Gastroesophageal reflux and respiratory phenomena in infants: status of the intraluminal impedance technique. J Pediatr Gastroente

5- Wenzl TG, Moroder C, Trachterna M, Thomson M, Silny J, Heimann G, Skopnik H: Esophageal pH monitoring and impedance measurement: a comparison of two diagnostic tests for gastroesophagea

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Dermatofibrosarcoma Protuberans

Dermatofibrosarcoma protuberans (DFSP) is a rare, low grade malignant soft tis sue tumor of the skin and subcutaneous tissue with a high propensity for local inv

Though mostly seen in middle age, a few cases have been described in infants and children between the ages of 14 months and 12 years. Clinically, DFSP present

firm nodular lesion fixed to the skin but mobile over the deep fascia, with a pattern of slow, progressive growth. Diagnosis is established after excisional biopsy or

common location of this tumor is the trunk followed by the extremities . CT-scan is useful to determine the tumor extent of penetration. DFSP has two histologic v

grade tumor, and a high-grade rare fibrosarcomatous variant demonstrating necrosis, high mitotic rate (> 10 mitose s high-power fie lds) and presence of pleomorp

associated with a poor clinical outcome. Metastasis is rare. Lung metastasis are most common, while lymph node metastasis is exceedingly. Rare. Wide surgical

three centimeters with inclusion of superficial fascia is currently the standard therapy in children. Mohs micrographic surgical excision has wide acce ptance amon

radiosensitive tumor.

References:

1- McKee PH, Fletcher CD: Dermatofibrosarcoma protub erans presenting in infancy and ch ildhood. J Cutan Pat hol 18(4):241-6, 1991

2- Keshen TH, Cederna PS, Savell VH Jr, Platz CE, Chang P, Ricciardelli EJ: Clinical and p atholog ical features of pediatric de rmatofibrosarcoma protu berans . Ann Plast Surg 35(6):633-7 , 1995

3- Pappo A S, Rao BN, Cain A, Bodn er S, Pratt CB: Dermatofibrosarcoma protub erans : the pediatric experience at St. Jude Children's Research Hospital. Pediatr Hematol Oncol 14(6):563-8, 1997

4- Bouyss ou-Gauthier ML, Labrous se F, Longis B, Bedane C, Bernard P, Bonnetblanc JM: Dermatofibrosarcoma prot uberans in childhood. Pediatr Dermatol 14(6):463-5, 19975- Mentzel T, Beham A, Katenkamp D, Dei Tos AP, Fletcher CD: Fibrosarcomatous ("high-grade") dermatofibrosarcoma protuberans: clinicopathologic and immunohistochemical study of a series of 41 c

significance. Am J Surg Pat hol 22(5):576-87, 19986- Marcus JR, Few JW, Seng er C, Reynolds M: Dermatofibrosarcoma prot uberans and th e Bednar tumor: treatment in the p ediatric population. J Ped iatr Surg 33(12):1811-4, 1998

7- Checketts SR, Hamilton TK, Baughman RD: Congenital and childhood d ermatofibrosarcoma protu berans : a case rep ort and review of the literature. J Am Acad Dermatol 42(5 Pt 2):907-13, 2000

8- Bowne WB, Antonescu CR, Leung DH, Katz SC, Hawkins WG, Woodruff JM, Brennan MF,

Lewis JJ: Dermatofibrosarcoma protuberan s: A clinicopathologic ana lysis of pat ients trea ted and followed at a single institution. Cancer 88(12):2711-20, 2000

Congenital Cystic Adenomatoid Malformation

Congenital Cystic Adenomatoid Malformation (CCAM) is a rare lung bud lesion characterized by the presence of a multicystic mass of immature pulmonary tissu

development of lobar and segmental bronchi. Embryologic origin is cessation of bronchiolar maturation with mesenchymal overgrowth occurring in the late 5th or

CCAM is divided into Type 1 (large, > 2 cm, irregular widely space cysts; the most common type), Type II (smaller, < 1 cm, closer together; more numerous cysts

bronchioles ), and Type III (large, solid appearance with small unrecognizable cyst). Due to communication between bronchiole-like structure and cyst significant

acute life-threatening respiratory distress (tachypnea, cyanosis and chest wall retraction) in one-third of cases. The other clinical presentation is late-onset pulmo

and dyspnea). Most cases have unilobar involvement. Bad prognostic factors associated with CCAM are fetal hydrops, polyhydramnios, increase mediastinal shi

presence of pulmonary hypoplasia. Postnatal diagnosis is confirmed with ches t CT-Scan. Symptomatic infants with CCAM should undergo immediate rese ction of

lobectomy). Limited lung resection (segmentectomy) preserves more functioning lung parenchyma and represents an alternative in bilobar disease. Since asympt

spontaneous regression, a policy of watchful observation with periodic imaging followed by elective resection is justified. Children with large lesions (> 50%) of th

significant chance of requiring surgical intervention. Better height and weight growth has been found in patients operated upon in later childhood compared with t

References:

1-Mentzer SJ, Filler RM, Phillips J: Limited pulmonary res ections for cong enital cys tic adeno matoid malformation of the lung. J Pediatr Su rg 27(11):1410-3, 1992

2- Dumez Y, Mandelbrot L, Radunovic N, Revillon Y, Dommergues M, Aubry MC, Aubry JP, Narcy F, Sonigo P: Prenatal management of congenital cystic adenomatoid malformation of the lung. J Pediat

3- Miller JA, Corteville JE, Langer JC: Congenital cys tic adeno matoid malformation in the fetus : natural history and p redictors of outcome. J Pediatr Surg 31(6):805-8, 1996

4- Kim WS, Lee KS, Kim IO, Suh YL, Im JG, Yeon KM, Chi JG, Han BK, Han MC: Congen ital cys tic adenomatoid malformation of th e lung: CT-pat hologic corre lation. AJR Am J Roentgenol 168(1):47-53,

5- Sapin E, Lejeune V, Barbet JP, Carricaburu E, Lewin F, Baron JM, Barbotin-Larrieu F, Helardot PG: Congenital aden omatoid diseas e of the lung: prenata l diagnosis and p erinatal management. Pediatr S

6- van Leeuwen K, Teitelbaum DH, Hirschl RB, Austin E, Adelman SH, Polley TZ, Marshall KW, Coran AG, Nugent C: Prenatal diagnosis of congenital cystic adenomatoid malformation and its postnata

and n atural hist ory. J Ped iatr Surg 34(5):794-8, 1999

7- Pinter A, Kalman A, Karsza L, Verebely T, Szemledy F: Long-term out come of co ngen ital cyst ic adeno matoid malformation. Pediatr Surg Int 15(5-6):332-5, 1999

8- Keidar S, Ben-Sira L, Weinberg M , Jaffa AJ, Silbiger A, Vinograd I: The pos tnatal management o f congen ital cystic aden omatoid malformation. Isr Med A ss oc J 3(4):258-61, 2001

Retching

Retching, choking and gagging are seve re, debilitating oral-motor dysfunctional complex symptoms seen rarely after fundoplication and gastrostomy placement i

children. Retching is not a symptom of reflux, but the first part of the ejection phase of the e metic reflex consisting of rhythmic contraction of the e ntire diaphrag

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wall muscles . The e tiology of this complex symptom is still not known. It is argued that following fundoplication gastric dysrhythmia ensues due to vagal afferent

injury, altered morphology or increased gastric filling. Initial management of postoperative retching includes antiemetics (dopamine or hydroxytryptamine recepto

domperidone or ondansetron. If symptoms persist, the child can be paced in continuous gastric feedings. With no improvement jejunal feedings bypassing the sto

vagal afferent stimulation results in significant relief. Children who retch preoperatively or have a hypersensitive emetic reflex has three times the risk of retchin

Retching can cause wrap migration or disruption. Development of a paraes ophageal hernia from failed crural repair is a surgical correctable cause of postoperati

fundoplication.

References:

1- Borowitz SM, Borowitz KC: Oral dys function following Nissen fundop lication. Dysph agia 7(4):234-7, 1992

2- Ravelli AM, Milla PJ: Vomiting an d gas troesophageal motor activity in children with disorde rs of t he cent ral nervous sys tem. J Pediatr Gast roenterol Nutr 26(1):56-63, 1998

3- Kimber C, Kiely EM, Spitz L: The failure rate o f surg ery for g astro -oeso phageal reflux. J Pediatr Surg 33(1):64-6, 1998

4- Richards CA, Andrews PL, Spitz L, Milla PJ: Nissen fund oplication may induce gas tric myoelectrical disturbance in children. J Ped iatr Surg 33(12):1801-5, 19985- Richards CA, Milla PJ, And rews PL, Spitz L: Retching and vomiting in neurologically impaired children afte r fundo plication: predictive preop erative facto rs. J Ped iatr Surg 36(9):1401-4, 2001

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