PEDIATRIC SURGERY UPDATE ©
VOLUME 19, 2002
Volume 19 No 01 JULY 2002
Laparoscopic Inguinal Herniography
Besides using the laparoscopic technique for identifying a contralateral
patent processus vaginalis using a trans-inguinal approach or repair a
recurrent inguinal hernia, the minimally invasive 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. Advantages
of the lap procedure 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, better cosmetic result, and 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 benefit is that there is no 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 likely. Disadvantages
include a longer procedure, higher recurrent rates, development of residual
hydrocele in males along with the complications attendant in violating
the abdominal cavity (vascular and bowel injury 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 repair of inguinal
hernia in infants and children.
References:
1- Schier F, Montupet P, Esposito C: Laparoscopic Inguinal
Herniography in Children: A Three-Center Experience with 933 repairs. J
Pediatr Surg 37(3): 395-397, 2002
2- Geisler DP, Jegathesan S, Parmley MC, McGee JM, Nolen
MG, Broughan TA: Laparoscopic exploration for the clinically undetected
hernia in infancy and childhood. 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 hernias in children: laparoscopic
aspects. Pediatr Surg Int 16(8):562-4, 2000
5- Schier F: Laparoscopic surgery of inguinal hernias
in children--initial experience. J Pediatr Surg 35(9):1331-5, 2000
6- Montupet P, Esposito C: Laparoscopic Treatment of
Congenital Inguinal Hernia in Children. J Pediatr 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 presence of a lethal associated anomaly (usually a cardiac defect),
central nervous system bleeding/injury from aggressive medical management
(i.e., ECMO), or iatrogenic barotrauma. Lethal pulmonary hypoplasia will
probably rest its solution in fetal intervention as more prenatal techniques
are used to identify the fetus with such a problem (progressive ipsilateral
pulmonary artery hypoplasia measure with fetal echo-doppler and fetal
lung volume measurement by magnetic resonance imaging correlates with progressive
fetal lung hypoplasia and worse postnatal outcomes). The recent postnatal
trends in management of babies suffering from CDH rest on three principles
that have been found to reduce mortality and morbidity: 1) use of soft
mechanical ventilation (spontaneous mechanical ventilation and minimal
inspiratory pressure settings) with permissive hypercapnia, 2) delay surgical
repair until hemodynamic, ventilatory and acid-base stabilization, 3) avoiding
ipsilateral chest tube placement since 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 Surg 37(3):
357-366, 2002
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 congenital diaphragmatic
hernia. Am J Obstet Gynecol 186(5 Pt 1):1085-1090, 2002
3- 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 diaphragmatic hernia.
BJOG 108(8):863-8, 2001
4- Kays DW, Langham MR, Ledbetter DJ, et al: Detrimental
Effects of Standard Medical Therapy in Congenital Diaphragmatic Hernia.
Ann Surg 230: 340-351, 1999
5- Serrano P, Reyes G, Lugo-Vicente HL: Congenital Diaphragmatic
Hernia: Mortality Determinants in a Hispanic Population. 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, pelvic tumor resection and spinal cord injury) condition. FI causes
a psychological, developmental and social stress of great magnitude. Assessment
of the problems includes the use of anal tonometry, electromyography and
pudendal nerve terminal motor latencies. Rectal emptying and angulation
can be evaluated with defecograms, MRI or scintigraphic proctography. The
management of FI is complex. For HD biopsychosocial treatment consisting
of explanation, extinction of fear and avoidance behavior, learning new
defecation behavior, learning an adequate straining technique, and generalization
toward daily life have been effective. Biofeedback is a harmless and inexpensive
treatment coordinating pelvic floor muscle contraction with the sensation
of rectal filling but has brought partial success in children with FI.
Other less effective forms of treatment are dynamic graciloplasty and sacral
nerve stimulation. Functional constipation associated with fecal incontinence
and the presence of segmental dilatation of the sigmoid or rectum responds
poorly to medical management. The megabowel lacks an adequate anorectal
reflex, shows decrease propulsive function causing a functional obstruction.
Segmental resection of the dilated segment has brought successful relief
of symptoms. Antegrade enemas by way of an appendicocecostomy or cecostomy
tube can achieve total colonic evacuation and socially controlled continence
in children with anorectal malformations and caudal agenesis in a high
percentage of cases.
References:
1- Darakhsan AA, Williams NS: Recent Innovations in the
Management of Fecal Incontinence. 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 Surg Int
16(5-6):312-6, 2000
3- Heymen S, Jones KR, Ringel Y, Scarlett Y, Whitehead
WE: Biofeedback treatment of fecal incontinence: 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 Pediatr Surg
37(1):76-9, 2002
5- Whitehead WE, Wald A, Norton NJ: Treatment options
for fecal incontinence. Dis Colon Rectum 44(1):131-42, 2001
6- Lee SL, Rowell S, Greenholz SK: Therapeutic cecostomy
tubes in infants with imperforate anus and caudal agenesis. J Pediatr Surg
37(3):345-7, 2002
7- Rintala RJ: Fecal incontinence in anorectal malformations,
neuropathy, and miscellaneous conditions. Semin Pediatr Surg 11(2):75-82,
2002
Volume 19 No 02 AUGUST 2002
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 probably an ovarian incarcerated inguinal hernia. On very
rare occasion the lump is a testis in a child with testicular feminization
syndrome. Clinically the irreducible ovarian lump is usually asymptomatic,
movable and non-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 incidence of ovarian torsion associated before repair.
Ovaries trapped within inguinal hernias undergo torsion far more commonly
than ovaries and tubes in the normal pelvic position increasing the chances
of infarction. 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 serves as a fixed point around which a twist
can occur. The risk of torsion and infarction creates the view that ovarian
hernias should be repaired at the earliest elective opportunity if they
can be reduced manually. Children with edema, tenderness or skin discoloration
in the inguinal area should be repaired immediately. Early recognition
and management of this condition reduce the risk of gonadal 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 wall of the hernial sac (sliding component).
References:
1- Goske MJ, Emmens RW, Rabinowitz R: Inguinal ovaries
in children demonstrated by high resolution real-time ultrasound. 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, Ratschek M, Schimpl G, Hollwarth ME: Ovary
in hernia sac: prolapsed or a descended gonad? J Pediatr Surg 34(6):977-80,
1999
4- Merriman TE, Auldist AW: Ovarian torsion in inguinal
hernias. Pediatr 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 colon and rectum after surgical diversion of the fecal
stream persisting indefinitely unless the excluded segment is reanastomosed.
Diversion colitis is characterized by rectal discomfort, pain, discharge,
tenesmus and bleeding. Symptoms occur three months or more after bowel
diversion. Diagnosis is established by colonic or rectal biopsy. Histologic
abnormalities included aphthous ulcers, crypt distortion, atrophy and abscesses,
a villous colonic surface, and a mixed acute and chronic inflammatory infiltrate
with patchy lymphoid hyperplasia. This condition is caused by the absence
of luminal short-chain fatty acids, the preferred metabolic substrates
of colonic epithelium. In children diversion colitis can be seen in bowel
derived Hirschsprung's disease, imperforate anus, ulcerative 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
mixture of short-chain fatty acids (propionate, acetate, butyrate) and/or
5-Aminosalicylic acid (5-ASA) to control symptoms. Definitive treatment
consists of excision of rectum or stomal closure.
References:
1- Harig JM, Soergel KH, Komorowski RA, Wood CM: Treatment
of diversion colitis with short-chain-fatty acid irrigation. N Engl J Med
320(1):23-8, 1989
2- Komorowski RA: Histologic spectrum 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, West AB:
Mucosal inflammation in pediatric diversion colitis: a quantitative analysis.
J Pediatr Gastroenterol Nutr 25(3):273-80, 1997
5- Kiely EM, Ajayi NA, Wheeler RA, Malone M: Diversion
Procto-Colitis: Response to Treatment with Short-Chain Fatty Acids. 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-bubble appearance of the dilated stomach and duodenal bulb seen
prenatally (US) as two anechoic cysts or after birth (simple abdominal
films) is characteristically diagnostic. DA is associated one-third of
the time with Down's syndrome followed by cardiac malformations (20%).
Prenatal karyotyping and fetal echocardiogram of suspected DA cases will
establish the association. Clinically, the child with DA presents with
bilious vomiting and epigastric distension (dilated stomach) depending
whether the atresia occurs proximally (10%) or distally (90%) to the papilla
of Vater. Bowel obstruction 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 is recommended. After correction
of electrolytes imbalances, management of DA consists of diamond-shaped
duodeno-duodenostomy. If the proximal duodenum is massively dilated a tapering
duodenoplasty (imbrication or stapler resection) will help reduce the possibilities
of a functional anastomotic obstruction. Late complications include motility
disorders, megaduodenum, gastroesophageal reflux, duodenal-gastric reflux,
gastritis, peptic ulcer disease, blind loop syndrome and biliary-pancreatic
conditions which may be observed months to years after surgical management.
References:
1- Ein SH, Kim PC, Miller HA: The late nonfunctioning
duodenal atresia 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):1197-9,
1999
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 bowel in
patients with duodenal atresia and stenosis. J Pediatr Surg 32(8):1157-60,
1997
4- Upadhyay V, Sakalkale R, Parashar K, Mitra SK, Buick
RG, Gornall P, Corkery JJ: Duodenal atresia: a comparison of three modes
of treatment. 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, 1993
6- Akhtar J, Guiney EJ: Congenital duodenal obstruction.
Br J Surg 79(2):133-5, 1992
7- Kimura K, Mukohara N, Nishijima E, Muraji T, Tsugawa
C, Matsumoto Y: Diamond-shaped anastomosis for duodenal atresia: an experience
with 44 patients over 15 years. J Pediatr Surg 25(9):977-9, 1990
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 common benign parotid tumor in children includes hemangioma, pleomorphic
adenoma and lymphangiomas. Infants with a rapidly enlarging violaceous
or soft tumor in the parotid region harbors a hemangioendothelioma. Hemangioendothelioma
is the most common parotid gland tumor of childhood. They seldom need excision
as spontaneous regression is the norm. An asymptomatic, slow growing solid
mass is the most common presentation in older children. Tenderness is associated
with an infectious process. Diagnosis includes the use of Doppler ultrasound,
CT-Scan, MRI and fine needle aspiration (FNA) biopsy. Salivary gland carcinoma
is rare in children. Most common histological type is mucoepidermoid either
as a primary neoplasm or secondary malignancy after neck irradiation. Management
consists of superficial or total parotidectomy extent which is selected
during the surgical procedure depending on deep gland or fascial nerve
involvement. The tumor must be widely excised. Recurrence is managed with
postoperative irradiation for high or intermediate grade malignancies.
Rhabdomyosarcoma arising in the parotid gland area is another locally invasive
aggressive tumor that presents early with swelling and symptoms of seventh
nerve deficit. This tumor needs surgical excision followed by chemotherapy
and irradiation.
References:
1- Zurrida S, Alasio L, Tradati N, Bartoli C, Chiesa
F, Pilotti S: Fine-needle aspiration of parotid masses. Cancer 72(8):2306-11,
1993
2- Rogers DA, Rao BN, Bowman L, Marina N, Fleming ID,
Schropp KP, Lobe TE: Primary malignancy of the salivary gland in children.
J Pediatr Surg 29(1):44-7, 1994
3- Khadaroo RG, Walton JM, Ramsay JA, Hicks MJ, Archibald
SD: Mucoepidermoid carcinoma of the parotid gland: a rare presentation
in a young child. J Pediatr Surg 33(6):893-5, 1998
4- Salomao DR, Sigman JD, Greenebaum E, Cohen MB: Rhabdomyosarcoma
presenting as a parotid gland mass in pediatric patients: fine-needle aspiration
biopsy findings. Cancer 84(4):245-51, 1998
5- Roebuck DJ, Ahuja AT: Hemangioendothelioma of the
parotid gland in infants: sonography and correlative MR imaging. AJNR Am
J Neuroradiol 21(1):219-23, 2000
6- Orvidas LJ, Kasperbauer JL, Lewis JE, Olsen KD, Lesnick
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 sporadically described. Lesions of the anogenital area have a
strong predilection for females. The clinical presentation can start with
an undiagnosed non-healing ulcer, a pale macule, erythematous papule, bruise
or port-wine 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 potentiating role in ulcer development. MRI helps determine
the extension of the hemangioma toward the pelvis and surrounding tissues.
Though most hemangiomas spontaneously involute over a period of years,
those located in the perineal and ano-rectal region are troublesome lesions
causing repeated ulcerations and subsequent pain. The subcutaneous perineal
hemangioma can be managed conservatively, with systemic steroids or using
alpha interferon therapy depending on the symptomatology of the child.
Diffuse cavernous hemangioma of the colon, rectum and anus can be managed
with initial temporary colostomy followed by modified endorectal pull-through.
References:
1- Liang MG, Fireden IJ: Perineal and Lip Ulceration
as the Presenting Manifestation of hemangioma of Infancy. Pediatrics 99(2):
256-259, 1997
2- Bouchard S, Yazbeck S, Lallier M: Perineal hemangioma,
anorectal malformation, and genital anomaly: a new association? 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):245-7, 1992
5- Achauer BM, Vander Kam VM: Ulcerated anogenital hemangioma
of infancy. Plast Reconstr Surg 87(5):861-6, 1991
Fetus-in-Fetu
Fetus-in-fetu is a very rare condition in which a malformed parasitic
twin is found inside the body of its partner as an abdominal fetiform calcified
mass. They are typically located in the retroperitoneum. Symptoms relate
to the mass effect and include abdominal distension, feeding difficulty,
vomiting and dyspnea. It represents an abortive attempt of identical twinning
where 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 supply to the former 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 composed of the three
germ cell 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 undergo organogenesis. 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. Chromosomal analysis of the fetus
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 WJ: Fetus in fetu:
a case 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, Abramowsky CR: Fetus-in-fetu with malignant recurrence. J Pediatr Surg
32(10):1476-9, 1997
3- Hoeffel CC, Nguyen KQ, Phan HT, Truong NH, Nguyen
TS, Tran TT, Fornes P: Fetus in fetu: a case report and literature review.
Pediatrics 105(6):1335-44, 2002
4- Mills P, Bornick PW, Morales WJ, Allen M, Gilbert-Barness
E, Johnson PK, Quintero R: Ultrasound prenatal diagnosis of fetus in fetu.
Ultrasound Obstet Gynecol 18(1):69-71, 2001
5- Federici S, Prestipino M, Domenichelli V, Antonellini
C,
Sciutti R, Domini R: Fetus in fetu: report of an additional, well-developed
case. Pediatr Surg 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 newborns. Colonic atresia (CA) can be associated with
distal skeletal deformities, imperforate anus, Hirschsprung's and abdominal
wall defects. Complicated cases are associated with partial or total hindgut
absence and severe deformities of the genitourinary tract (bladder and
cloacal exstrophy). Colonic Atresia is the result of an intrauterine mesenteric
vascular accident. Clinically they show abdominal distension, bilious vomiting
and obstipation. Simple abdominal films shows hugely distended distal bowel
loops and air-fluid levels. Barium enema will show a microcolon. Operative
intervention is urgent since a competent ileo-cecal valve makes the obstruction
a close loop. During surgery patency of the gastrointestinal tract must
be confirmed distally to the atresia to avoid missing a second associated
atresia. For atresias located in the right colon, resection and end to
oblique primary anastomosis is recommended. Atresias located in the left
colon, critically ill children or complex combinations of absent hindgut
should be initially managed with a diverting colostomy. Suspect the coexistence
of Hirschsprung's disease and colon atresia when a functional obstruction
is present after repair of the atresia. Suction rectal biopsy before closing
the colostomy should solve this problem. Prognosis depends on type of surgery
and associated malformations.
References:
1- Coran AG, Eraklis AJ: Atresia of the colon. Surgery
65: 828-831, 1969
2- Powell RW, Raffensperger JG: Congenital Colonic Atresia.
J Pediatr Surg 17(2): 166-170, 1982
3- Pohlson EC, Hatch EI Jr, Glick PL, Tapper D: Individualized
management of colonic atresia. Am J Surg 155(5):690-2, 1988
4- Davenport M, Bianchi A, Doig CM, Gough DC: Colonic
atresia: current results of treatment. J R Coll Surg Edinb 35(1):25-8,
1990
5- Williams MD, Burrington JD: Hirschsprung's disease
complicating colon atresia. J Pediatr Surg 28(4):637-9, 1993
6- Dalla Vecchia LK, Grosfeld JL, West KW, Rescorla FJ,
Scherer LR, Engum SA: Intestinal atresia and stenosis: a 25-year experience
with 277 cases. Arch Surg 133(5):490-6, 1998
7- Akgur FM, Olguner M, Hakguder G, Ozer E, Aktug T:
Colonic atresia associated with Hirschsprung's disease: it is not a diagnostic
challenge. Eur J Pediatr Surg 8(6):378-9, 1998
8- Karnak I, Ciftci AO, Senocak ME, Tanyel FC, Buyukpamukcu
N: Colonic atresia: surgical management and outcome. 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 congenital (stenosis), develop in children receiving high dose
pancreatic enzyme supplement (cystic fibrosis) or after inflammatory bowel
disease. Post-NEC strictures occur both after medical and surgical management.
In medically-treated infants symptoms of bowel obstruction usually
begin six to eight weeks after resolution of NEC, while surgically managed
infants develop asymptomatic strictures distal to an enterostomy. The clinical
manifestations of colonic strictures include abdominal distention, bilious
vomiting, hematochezia, diarrhea, disaccharide intolerance, chronic gastrointestinal
blood 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 diagnosis in symptomatic cases. Although routine studies
have no advantage over clinical follow-up, some studies propose a diagnostic
protocol using an early upper GI study with non-ionic water soluble contrast
material followed by a contrast enema in suspicious cases as a reliable
method of diagnosing strictures. Contrast enemas are essential prior to
restoring bowel continuity after surgery. Balloon catheter dilatation of
the stricture has been tried but recurrence closure rates are too high
to be recommended as definite therapy. Once identified management consists
of resection and primary anastomosis of the stricture.
References:
1- Kosloske AM, Burstein J, Bartow SA: Intestinal obstruction
due to colonic stricture following neonatal necrotizing enterocolitis.
Ann Surg 192(2):202-7, 1980
2- Ball WS Jr, Seigel RS, Goldthorn JF, Kosloske AM:
Colonic strictures in infants following intestinal ischemia. Treatment
by balloon catheter dilatation. Radiology 149(2):469-71, 1983
3- Hartman GE, Drugas GT, Shochat SJ: Post-necrotizing
enterocolitis strictures presenting with sepsis or perforation: risk of
clinical observation. J Pediatr Surg 23(6):562-6, 1988
4- Nanjundiah P, Lifschitz CH, Gopalakrishna GS, Cochran
WJ, Klish WJ: Intestinal strictures presenting with gastrointestinal blood
loss. J Pediatr Surg 24(2):174-6, 1989
5- Radhakrishnan J, Blechman G, Shrader C, Patel MK,
Mangurten HH, McFadden JC: Colonic strictures following successful medical
management of necrotizing enterocolitis: a prospective study evaluating
early gastrointestinal contrast studies. J Pediatr Surg 26(9):1043-6,
1991
6- Collins MH, Azzarelli B, West KW, Chong SK, Maguiness
KM, Stevens JC: Neuropathy and vasculopathy in colonic 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 colon. They all share the same pathogenetic mechanism:
a long freely redundant mobile colon and mesocolon, lack of fixation and
short mesenteric attachment of the proximal and distal mesocolic limbs.
Colonic volvulus have been associated to chronic constipation, high fiber
diet, mental retardation and Hirschsprung's disease. Central abdominal
pain of sudden onset followed by abdominal distension, tenderness, bloody
mucoid discharge or inability to pass flatus is characteristic. Simple
abdominal films will show large bowel dilated loops with air-fluid levels.
Barium enema 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 operative since colonoscopy or 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 limited segmental
disease and colopexy for extensive bowel involvement.
References:
1- Mellor MF, Drake DG: Colonic volvulus in children:
value of barium enema for diagnosis and treatment in 14 children. AJR Am
J Roentgenol 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. Aust N Z J Surg 70(4):258-62, 2000
4- Mercado-Deane MG, Burton EM, Howell CG: Transverse
colon volvulus in pediatric patients. Pediatr Radiol 25(2):111-2,
1995
5- Houshian S, Sorensen JS, Jensen KE: Volvulus of the
transverse colon in children. J Pediatr Surg 33(9):1399-401, 1998
6- Salas S, Angel CA, Salas N, Murillo C, Swischuk L:
Sigmoid volvulus in children and adolescents. J Am Coll Surg 190(6):717-23,
2000
7- Samuel M, Boddy SA, Capps S: Volvulus of the transverse
and sigmoid colon. Pediatr Surg Int 16(7):522-4, 2000
Volume 19 No 05 NOVEMBER 2002
Esophago-Gastric Dissociation
Gastroesophageal reflux is a common and distressing problem in neurologically
affected children. Couple with diminished salivary clearance, pharyngo-esophageal
incoordination and esophageal dysmotility the incidence of pulmonary aspiration
and infection is increased in this pediatric population. Fundoplication
and gastrostomy are effective in providing nutrition, reducing aspiration
and improving quality of care. Unfortunately, a large number of these kids
develop complications after the fundoplication. They include: wrap herniation
into chest (entire or paraesophageal), disruption, gagging, retching and
recurrent preop GER symptoms (vomiting and pneumonia are the most troublesome).
Theories explaining this high rate of failure are: supine position, swallowing
incoordination, esophageal dysmotility, spasticity, seizures, delayed gastric
emptying, chronic constipation and scoliosis (increase intraabdominal pressure).
Recurrent reflux can be managed with redo fundoplication, though postoperative
complications increase significantly. A viable alternative is esophago-gastric
dissociation (EGD). EGD consists of closing the esophagogastric junction
and establishing bowel continuity with a Roux-en-y esophagojejunostomy
and an end-to-side jejuno-jejunostomy while feeding the child through the
gastrostomy along with unrestricted oral intake. The procedure allows
free passage of saliva avoiding the obstructive nature of a fundoplication
(tight wrap) in a child with esophageal dysmotility. EGD is indicated in
children with pathological reflux and severely neurological disorders,
poor or none oral feeding and difficult to manage pharyngo-esophageal incoordination
and esophageal dysmotility.
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 impaired versus
neurologically normal children. J Pediatr Surg 25(11):1169-73, 1990
2- Bianchi A: Total Esophagogastric Dissociation: An
Alternative Approach. J Pediatr Surg 32(9): 1291-1294, 1997
3- Danielson PD, Emmens RW: Esophagogastric Disconnection
for Gastroesophageal Reflux in Children with Severe Neurological Impairment.
J Pediatr Surg 34(1): 84-87, 1999
4- Gatti C, Federici di Abriola G, Villa M, De Angelis
P, Laviani R, La Sala E, Dall'Oglio L: Esophagogastric Dissociation versus
Fundoplication: Which is Best for Severely Neurologically Impaired Children.
J Pediatr Surg 36(5): 677-680, 2001
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. Testicular torsion (TT) is the most common pediatric urological
emergency. TT can occur extravaginally or intravaginally. Extravaginal
TT is a perinatal event (in utero) which occurs when the spermatic cord
undergoes 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 exploration will yield a viable testis. 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 attachment of the tunica 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 most frequently in adolescents.
Child develops sudden scrotal pain, edema, nausea and vomiting. Cremasteric
reflex is lost. Color Doppler sonography (readily available and non-invasive)
in experienced hands or Technetium scan are both reliable in suggesting
the diagnosis. Immediate scrotal exploration is the best means available
of establishing the diagnosis and saving a testis in TT. Whether you remove
a dead testis or relieve the torsion the contralateral testis should also
be pexed.
References:
1- Hermann D: The pediatric acute scrotum. Pediatr Ann
18(3):198-202, 1989
2- Cilento BG, Najjar SS, Atala A: Cryptorchidism and
testicular torsion. Pediatr Clin North Am 40(6):1133-49, 1993
3- Kass EJ, Lundak B: The acute scrotum. 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
5- Burgher SW: Acute scrotal pain. Emerg Med Clin North
Am 16(4):781-809, 1998
6- Munden MM, Trautwein LM: Scrotal pathology in pediatrics
with sonographic imaging. Curr Probl Diagn Radiol 29(6):185-205,
2000
Alkaline Reflux
Alkaline (pH > 7) gastroesophageal reflux (AGER) is not so rare in children
affecting 10% of all refluxers. Acid reflux is more common (50%), followed
by combined acid-alkaline reflux (20%). AGER causes gastritis and esophagitis
and is associated with repetitive vomiting, excessive crying and chronic
respiratory disease. In an alkaline environment trypsin and deconjugated
bile salts produce the greatest injury to the esophageal mucosa. Simultaneous
gastroesophageal pH monitoring can help establish the diagnosis. Medication
can be specifically 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 procedure and gastropyloric dysfunction might
continue with dyspeptic symptoms and gastritis needing further therapy.
The aim is guide clinicians to choose efficient anti-reflux therapy based
on the type of reflux.
References:
1- Vandenplas Y, Loeb H: Alkaline gastroesophageal reflux
in infancy. J Pediatr Gastroenterol Nutr 12(4):448-52, 1991
2- Malthaner RA, Newman KD, Parry R, Duffy LF, Randolph
JG: Alkaline gastroesophageal reflux in infants and children. J Pediatr
Surg 26(8):986-90, 1991
3- Tovar JA, Wang W, Eizaguirre I: Simultaneous gastroesophageal
pH monitoring and the diagnosis 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 Gastroenterol
Nutr 28(4):423-8, 1999
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 gastroesophageal reflux. J Pediatr
Gastroenterol Nutr 34(5):519-23, 2002
Volume 19 No 06 DECEMBER 2002
Dermatofibrosarcoma Protuberans
Dermatofibrosarcoma protuberans (DFSP) is a rare, low grade malignant
soft tissue tumor of the skin and subcutaneous tissue with a high propensity
for local invasion and recurrence. 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 presents as an asymptomatic
raised, 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 punch biopsy. The most 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 variants: the
more typical low-grade tumor, and a high-grade rare fibrosarcomatous variant
demonstrating necrosis, high mitotic rate (> 10 mitoses high-power fields)
and presence of pleomorphic areas. This last variant is associated with
a poor clinical outcome. Metastasis is rare. Lung metastasis are most common,
while lymph node metastasis is exceedingly. Rare. Wide surgical excision
using a margin of three centimeters with inclusion of superficial fascia
is currently the standard therapy in children. Mohs micrographic surgical
excision has wide acceptance among adults cases. DFSP is a radiosensitive
tumor.
References:
1- McKee PH, Fletcher CD: Dermatofibrosarcoma protuberans
presenting in infancy and childhood. J Cutan Pathol 18(4):241-6,
1991
2- Keshen TH, Cederna PS, Savell VH Jr, Platz CE, Chang
P, Ricciardelli EJ: Clinical and pathological features of pediatric dermatofibrosarcoma
protuberans. Ann Plast Surg 35(6):633-7 , 1995
3- Pappo AS, Rao BN, Cain A, Bodner S, Pratt CB: Dermatofibrosarcoma
protuberans: the pediatric experience at St. Jude Children's Research Hospital.
Pediatr Hematol Oncol 14(6):563-8, 1997
4- Bouyssou-Gauthier ML, Labrousse F, Longis B, Bedane
C, Bernard P, Bonnetblanc JM: Dermatofibrosarcoma protuberans in childhood.
Pediatr Dermatol 14(6):463-5, 1997
5- 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 cases with emphasis on
prognostic significance. Am J Surg Pathol 22(5):576-87, 1998
6- Marcus JR, Few JW, Senger C, Reynolds M: Dermatofibrosarcoma
protuberans and the Bednar tumor: treatment in the pediatric population.
J Pediatr Surg 33(12):1811-4, 1998
7- Checketts SR, Hamilton TK, Baughman RD: Congenital
and childhood dermatofibrosarcoma protuberans: a case report 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 protuberans: A clinicopathologic
analysis of patients treated 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 tissue due to abnormal development of lobar and segmental bronchi.
Embryologic origin is cessation of bronchiolar maturation with mesenchymal
overgrowth occurring in the late 5th or 6th weeks of gestation. 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 resembling dilated bronchioles), and Type III (large, solid appearance
with small unrecognizable cyst). Due to communication between bronchiole-like
structure and cyst significant air trapping can occur causing acute life-threatening
respiratory distress (tachypnea, cyanosis and chest wall retraction) in
one-third of cases. The other clinical presentation is late-onset pulmonary
infection (fever, cough and dyspnea). Most cases have unilobar involvement.
Bad prognostic factors associated with CCAM are fetal hydrops, polyhydramnios,
increase mediastinal shift, large size of the mass and presence of pulmonary
hypoplasia. Postnatal diagnosis is confirmed with chest CT-Scan. Symptomatic
infants with CCAM should undergo immediate resection of the lung lesion
(usually lobectomy). Limited lung resection (segmentectomy) preserves more
functioning lung parenchyma and represents an alternative in bilobar disease.
Since asymptomatic lesions can undergo spontaneous regression, a policy
of watchful observation with periodic imaging followed by elective resection
is justified. Children with large lesions (> 50%) of the hemithorax) has
a significant chance of requiring surgical intervention. Better height
and weight growth has been found in patients operated upon in later childhood
compared with those operated in infancy.
References:
1-Mentzer SJ, Filler RM, Phillips J: Limited pulmonary
resections for congenital cystic adenomatoid malformation of the lung.
J Pediatr Surg 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 Pediatr Surg 28(1):36-41,
1993
3- Miller JA, Corteville JE, Langer JC: Congenital cystic
adenomatoid malformation in the fetus: natural history and predictors 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: Congenital cystic adenomatoid malformation of the lung:
CT-pathologic correlation. AJR Am J Roentgenol 168(1):47-53, 1997
5- Sapin E, Lejeune V, Barbet JP, Carricaburu E,
Lewin F, Baron JM, Barbotin-Larrieu F, Helardot PG: Congenital adenomatoid
disease of the lung: prenatal diagnosis and perinatal management. Pediatr
Surg Int 12(2-3):126-9, 1997
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 postnatal presentation,
surgical indications, and natural history. J Pediatr Surg 34(5):794-8,
1999
7- Pinter A, Kalman A, Karsza L, Verebely T, Szemledy
F: Long-term outcome of congenital cystic adenomatoid 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 postnatal management of congenital cystic adenomatoid
malformation. Isr Med Assoc J 3(4):258-61, 2001
Retching
Retching, choking and gagging are severe, debilitating oral-motor dysfunctional
complex symptoms seen rarely after fundoplication and gastrostomy placement
in neurologically impaired children. Retching is not a symptom of reflux,
but the first part of the ejection phase of the emetic reflex consisting
of rhythmic contraction of the entire diaphragm and anterior abdominal
wall muscles. The etiology of this complex symptom is still not known.
It is argued that following fundoplication gastric dysrhythmia ensues due
to vagal afferent damage, gastric mucosal injury, altered morphology or
increased gastric filling. Initial management of postoperative retching
includes antiemetics (dopamine or hydroxytryptamine receptors antagonists)
such as domperidone or ondansetron. If symptoms persist, the child can
be paced in continuous gastric feedings. With no improvement jejunal
feedings bypassing the stomach to minimize gastric vagal afferent stimulation
results in significant relief. Children who retch preoperatively or have
a hypersensitive emetic reflex has three times the risk of retching after
fundoplication. Retching can cause wrap migration or disruption. Development
of a paraesophageal hernia from failed crural repair is a surgical correctable
cause of postoperative retching after fundoplication.
References:
1- Borowitz SM, Borowitz KC: Oral dysfunction following
Nissen fundoplication. Dysphagia 7(4):234-7, 1992
2- Ravelli AM, Milla PJ: Vomiting and gastroesophageal
motor activity in children with disorders of the central nervous system.
J Pediatr Gastroenterol Nutr 26(1):56-63, 1998
3- Kimber C, Kiely EM, Spitz L: The failure rate of surgery
for gastro-oesophageal reflux. J Pediatr Surg 33(1):64-6, 1998
4- Richards CA, Andrews PL, Spitz L, Milla PJ: Nissen
fundoplication may induce gastric myoelectrical disturbance in children.
J Pediatr Surg 33(12):1801-5, 1998
5- Richards CA, Milla PJ, Andrews PL, Spitz L: Retching
and vomiting in neurologically impaired children after fundoplication:
predictive preoperative factors. J Pediatr Surg 36(9):1401-4, 2001