PEDIATRIC SURGERY UPDATE ©
VOLUME 34, 2010
PSU Volume 34 No 01 JANUARY 2010
Emailing patients
With the advent of the Internet,
emailing has become one of the most powerful tools of communications.
It is relatively inexpensive, fast and text/image attachments can be
added. Electronic communication by way of emailing with patients is
used by 20% of physicians. Patient e-mail can potentially overload
physicians with extra work while health regulations can create concern
over electronic privacy issues. Requirements to ensure that
authenticity, confidentiality and integrity of the information
exchanged between the physician and the patient must be warrant.
Communications that are usually appropriate for e-mail include routine
appointment's requests, billing questions, routine prescription refill
requests, provision of follow-up information, and chronic disease
management questions. These policies can be provided to patients in
written form to help adhere and understand the appropriate use of
emails. Acute medical evaluation through email should be discouraged.
HIPAA requires encryption and appropriate protection (firewall) when
sending protected health information over the Net. Physicians planning
to incorporate electronic communication with their patients must be
prepared to manage unsolicited e-mail, maintain patient
confidentiality, and adopt practices that maximize the use of online
resources to enhance patient education. E-mail has the potential to
improve the doctor-patient relationship as a result of better
communication.
References:
1- Gerstle RS; American Academy of Pediatrics Task Force on Medical
Informatics: E-mail communication between pediatricians and their
patients. Pediatrics. 114(1):317-21, 2004
2- Biermann JS, Golladay GJ, Peterson RN: Using the internet to enhance
physician-patient communication. J Am Acad Orthop Surg. 14(3):136-44,
2006
3- Brooks RG, Menachemi N: Physicians' use of email with patients: factors influencing electronic
communication and adherence to best practices. J Med Internet Res. 24;8(1):e2, 2006
4- O'Brien JA: Netiquette: e-mail for group practices. J Med Pract Manage. 22(4):201-3, 2007
5- Cohall A, Hutchinson C, Nye A: Secure e-mail applications:
strengthening connections between adolescents, parents, and health
providers. Adolesc Med State Art Rev. 18(2):271-92, 2007
6- Lang KT 2nd, Kiel JM: An analysis of the utilization of e-mail by
physicians and patients: defining benefits, addressing concerns and
forecasting implications on the doctor-patient relationship. J Healthc
Inf Manag. 22(2):27-32, 2008
Suction Rectal Biopsy
Suction rectal biopsy (SRB) using a
capsule with a side hole has been present as an important diagnostic
tool since the late 60s. The procedure is simple and a suction rectal
biopsy tool is available. The capsule hook to a shaft and pistol is
introduced through the rectum into the anus until the side hole of the
capsule is 1.5 to 2 cm above the dentate line. By means of tubing a
vacuum gauge connected to the shaft and using a 60 mL syringe suction
is exerted so that the mucosa/submucosa adheres to the side hole of the
capsule. The pistol is slowly fired cutting the small fragment of
specimen caught inside the side hole of the capsule. At least three
specimens (posterior and lateral wall) should be taken to have
representative specimens so that the pathologist can have submucosa in
at least 15 sections. SRB is indicated in babies with delayed passage
of meconium, bowel obstruction, bowel dysmotility and/or history of
constipation when Hirschsprung's disease or other dysganglionosis. No
anesthesia is necessary and there have been very few reported
complications including bleeding and rectal perforation. The instrument
can be completely dismantled, physically cleaned and heat or chemically
sterilized. Children aged six months or older can benefit from using a
rectal cup biopsy forceps, nasal cutting forceps or an open biopsy
under general anesthesia.
References:
1- Weintraub WH, Heidelberger KP, Coran AG: A simplified approach to
diagnostic rectal biopsy in infants and children. Am J Surg.
134(2):307-10, 1977
2- Andrassy RJ, Isaacs H, Weitzman JJ: Rectal suction biopsy for the
diagnosis of Hirschsprung's disease. Ann Surg. 193(4):419-24, 1981
3- Freeman JK: A new instrument for suction rectal biopsy in the
diagnosis of Hirschsprung's disease. Pediatr Surg Int. 12(1):76-7, 1997
4- Alizai NK, Batcup G, Dixon MF, Stringer MD: Rectal biopsy for
Hirschsprung's disease: what is the optimum method? Pediatr Surg Int.
13(2-3):121-4, 1998
5- Kobayashi H, Li Z, Yamataka A, Lane GJ, Miyano T: Rectal biopsy:
what is the optimal procedure? Pediatr Surg Int. 18(8):753-6, 2002
6-Montedonico S, Piotrowska AP, Rolle U, Puri P: Histochemical staining
of rectal suction biopsies as the first investigation in patients with
chronic constipation. Pediatr Surg Int. 24(7):785-92, 2008
Vulvar Lipomas
Lipoma is the most common soft tissue
tumor consisting of mature fat cells and mainly located in the
subcutaneous tissue. Most locations are the head and neck, trunk and
extremity. Finding lipomas in the area of the genitalia is very
rare in infants and children. This is specially true for vulvar lipomas
in girls. Vulvar lipomas are soft well-demarcated or pedunculated
slowly growing swelling that appears in the labial region of girls.
They ranged in size from 2.0 to 8.0 cm in maximum dimensions. Usually
the child is around ten years of age and the right vulva is affected
more commonly then than the left. The differential diagnosis includes
an inguinal hernia, soft-tissue tumor (granular cell tumor),
hemangioma, lymphangioma or Bartholin cyst. Ultrasound (nonspecific
homogenous masses with lobular structures consistent with fat
deposition), computed tomography and magnetic resonance imaging are
useful in diagnosing lipomas and differentiating them from vulvar cysts
or inguinal hernias. Vulval lipomas have a benign clinical course,
though they keep growing. The treatment of choice is complete surgical
excision under general anesthesia as a outpatient procedure.
References:
1- Dewhurst CJ: Tumors of the genital tract in childhood and adolescence. Clin Obstet Gynecol. 20(3):595-606, 1977
2- Cohen Z, Kapuller V, Maor E, Mares AJ: Granular cell tumor
(myoblastoma) of the labia major: a rare benign tumor in childhood. J
Pediatr Adolesc Gynecol. 12(3):155-6, 1999
3- Iwasa Y, Fletcher CD: Distinctive prepubertal vulval fibroma: a
hitherto unrecognized mesenchymal tumor of prepubertal girls: analysis
of 11 cases. Am J Surg Pathol. 28(12):1601-8, 2004
4- Oh JT, Choi SH, Ahn SG, Kim MJ, Yang WI, Han SJ: Vulvar lipomas in
children: an analysis of 7 cases. J Pediatr Surg. 44(10):1920-3, 2009
PSU Volume 34 No 02 FEBRUARY 2010
Bell's Palsy
Bell's palsy is an acute idiopathic
paralysis of the VII cranial nerve (facial nerve) resulting in
inability to control facial muscles on the affected side. Bell's
palsy is a non life-threatening disorder that can cause
significant impact in the life of the child. Other conditions that can
cause fascial paralysis include brain tumor, head trauma, meningitis
and Lyme's disease. The mean age of involvement is eight years with a
similar sex predilection. It is thought that an inflammatory condition
leads to swelling of the facial nerve and compression in the narrow
bone canal leading to nerve inhibition, damage or death. No readily
identifiable cause for Bell's palsy has been found, though acute immune
demyelination triggered by a viral infection may be responsible. The
school nurse may be the first person to assess facial palsy and muscle
weakness in children. Careful diagnosis and workup using head CT and
MRI is warrant in all cases to exclude other serious causes of fascial
paralysis. MRI enhancement of the distal intrameatal and labyrinthine
segments of the nerve canal are specific for facial nerve palsy.
Complete spontaneous recovery is seen in almost 70% of patients within
six weeks of onset, but up to 30% have delayed or incomplete recovery.
Use of steroid or antiviral therapy is controversial since most
children recover without treatment. Physiotherapy, in the form of
transcutaneous peripheral nerve stimulation, has an important role.
References:
1- Kinoshita T, Ishii K, Okitsu T, Okudera T, Ogawa T: Facial nerve
palsy: evaluation by contrast-enhanced MR imaging. Clin Radiol.
56(11):926-32, 2001
2- Dhiravibulya K: Outcome of Bell's palsy in children. J Med Assoc Thai. 85(3):334-9, 2002
3- Singhi P, Jain V: Bell's palsy in children. Semin Pediatr Neurol. 10(4):289-97, 2003
4- Atzema C, Goldman RD: Should we use steroids to treat children with Bell's palsy? Can Fam Physician. 52:313-4, 2006
5-Shih WH, Tseng FY, Yeh TH, Hsu CJ, Chen YS: Outcomes of facial palsy in children.Acta Otolaryngol. 15:1-6, 2008
6- Gordon SC: Bell's palsy in children: role of the school nurse in
early recognition and referral. J Sch Nurs. 24(6):398-406, 2008
Shoulder Dislocation
Shoulder dislocation in infants most
commonly occurs in the newborn period, directly after delivery. Breech
presentation, macrosomia, prolonged labor and assisted birth are risk
factors associated with shoulder dystocia. Shoulder dystocia associated
with brachial plexus palsy occurs in approximately 1.5% of all births.
Associated with brachial plexus palsy, posterior shoulder dislocation
and subluxation can develop directly after birth or even one year later
due to the glenohumeral deformity that results from the persistence
muscle imbalance. Mean age at time of diagnosis is six months.
Diagnosis of shoulder dislocation is based in physical examination,
radiographs, ultrasound and MRI. Specific clinical signs include
asymmetry of skin folds of the axilla or the proximal aspect of the
arm, apparent shortening of the humeral segment, a palpable asymmetric
fullness in the posterior region of the shoulder, or a palpable click
during shoulder manipulation. All children with a brachial plexus birth
lesion should be screened, above the assessment of neurological
recovery, during the first year of life for posterior shoulder
dislocation since such a condition may occur in 7% of children
with a brachial plexus birth lesion. Management consists of prompt
closed reduction of the shoulder by traction combined with adduction of
the upper arm and countertraction with a gauze encircling the chest.
The functional outcome is related to the severity of the neurological
lesion, the duration of the dislocation and onset of deformity.
References:
1- Dodds SD, Wolfe SW: Perinatal brachial plexus palsy. Curr Opin Pediatr. 2000 Feb;12(1):40-7.
2- Moukoko D, Ezaki M, Wilkes D, Carter P: Posterior shoulder
dislocation in infants with neonatal brachial plexus palsy. J Bone
Joint Surg Am. 2004 Apr;86-A(4):787-93
3- Kambhampati SB, Birch R, Cobiella C, Chen L: Posterior subluxation
and dislocation of the shoulder in obstetric brachial plexus palsy. J
Bone Joint Surg Br. 2006 Feb;88(2):213-9
4- Dahlin LB, Erichs K, Andersson C, Thornqvist C, Backman C,
Düppe H, Lindqvist P, Forslund M: Incidence of
early posterior shoulder dislocation in brachial plexus birth
palsy. J Brachial Plex Peripher Nerve Inj. 2007 Dec 16;2:24.
5- Wessels R, Sleeboom C, de Vries JI, van Elburg RM: Shoulder
dislocation in a very-low-birth-weight infant: case report and review
of the literature. J Pediatr Surg. 2009 Dec;44(12):e19-20
Yolk Sac Tumor
Yolk sac tumor, also known as
endodermal sinus tumor, is a common phenotype of testicular malignancy
arising in descended testes. This tumor does not arise from the yolk
sac, as the name implies, but from the germinal epithelium of the
testis which has the ability to mimic the development pattern
found in the yolk sac. Painless unilateral testicular enlargement is
the most common presenting symptom in children. An associated hydrocele
can delay the correct diagnosis. Ultrasound and CT scan will help
suggest the diagnosis and extension of tumor. The definitive diagnostic
step is surgical exposure of the testis through an inguinal incision,
vascular exclusion and high cord ligation removal for histologic
examination. Microscopy reveals an intermingle of epithelial and
mesenchymal elements in a characteristic embryoid organoid pattern.
Yolk sac tumor secretes alpha feto-protein, a marker helpful in the
follow-up of these patients. The tumor spreads to lymph nodes and
hematogenous (lung). After surgery the need of chemotherapy,
radiotherapy or retroperitoneal lymph node dissection will depend on
the presence of residual micro- or macroscopic disease. Patients with
localized disease and normal postoperative serum alpha-fetoprotein
levels do not benefit from retroperitoneal node dissection,
postoperative abdominal irradiation or chemotherapy. Patients with
retroperitoneal nodal involvement, widely metastatic disease or
recurrent disease can be treated successfully with chemotherapy and in
some cases with radiation therapy. For the few who do relapse,
treatment at the time of relapse is curative for the majority.
References:
1- Olsen MM, Raffensperger JG, Gonzalez-Crussi F, Luck SR, Kaplan WE,
Morgan ER: Endodermal sinus tumor: a clinical and pathological
correlation. J Pediatr Surg. 17(6):832-40, 1982
2- Flamant F, Nihoul-Fekete C, Patte C, Lemerle J: Optimal treatment of
clinical stage I yolk sac tumor of the testis in children. J Pediatr
Surg. 21(2):108-11, 1986
3- Griffin GC, Raney RB Jr, Snyder HM, Duckett J, D'Angio GJ: Yolk sac
carcinoma of the testis in children. J Urol. 137(5):954-7, 1987
4- Shah JP, Kumar S, Bryant CS, Ali-Fehmi R, Malone JM Jr, Deppe G,
Morris RT: A population-based analysis of 788 cases of yolk sac tumors:
A comparison of males and females. Int J Cancer. 123(11):2671-5, 2008
5- De Backer A, Madern GC, Wolffenbuttel KP, Oosterhuis JW,
Hakvoort-Cammel FG, Hazebroek FW: Testicular germ cell tumors in
children: management and outcome in a series of 20 patients. J Pediatr
Urol. 2(3):197-201, 2006
6- Steffens J, Treiyer A, Calaminus G: Management of pediatric
testicular tumors : diagnosis, therapy, and follow-up. Urologe A.
48(4):359-63, 2009
PSU Volume 34 No 03 MARCH 2010
Tracheobronchial Remnants
Tracheobronchial remnants (TBR) are a
rare but consistent cause of congenital esophageal stenosis in newborns
and infants. The stenosis occurs in the lower esophagus (distal third).
TBR anomalies including the cartilaginous rest sometimes present are
viewed as different manifestations of a spectrum of abnormal embryonic
separation of the foregut from the respiratory tract occurring during
the 25th day of gestation. Histologically, the TBR includes cartilage,
early columnar metaplasia, disorganized/hyperplastic muscular layer and
ectopic glandular tissue. The most common association in children with
TBR is the presence of concomitant esophageal atresia a/o
tracheoesophageal fistula. The differential diagnosis includes peptic
acid esophageal stricture due to gastroesophageal reflux. Symptoms
start in early infancy includes progressive dysphagia and vomiting of
solid food, food impaction, stridor, repeated respiratory infection,
and failure to thrive. Esophagogram shows a narrow stricture in the
lower esophagus with proximal dilatation. The mucosa is normal during
endoscopy with biopsy. Esophageal dilatation is not very effective and
carries a high risk of perforation and leakage. Surgical resection of
the stenotic segment with end-to-end anastomosis is the treatment of
choice. Resection can be performed through either laparotomy or
thoracotomy depending on the location of the lesion. An antireflux
operation concomitant with the resection of the remnant will help
prevent gastroesophageal reflux in children with a narrowing near the
esophagogastric junction.
References:
1- Yeung CK, Spitz L, Brereton RJ, Kiely EM, Leake J: Congenital
esophageal stenosis due to tracheobronchial remnants: a rare but
important association with esophageal atresia. J Pediatr Surg.
27(7):852-5, 2992
2- Olguner M, Ozdemir T, Akgür FM, Aktuğ T:
Congenital esophageal stenosis owing to tracheobronchial remnants: a
case report. J Pediatr Surg. 32(10):1485-7, 1997
3- Dutta HK, Mathur M, Bhatnagar V: A histopathological study of
esophageal atresia and tracheoesophageal fistula. J Pediatr Surg.
35(3):438-41, 2000
4- Kawahara H, Imura K, Yagi M, Kubota A: Clinical characteristics of
congenital esophageal stenosis distal to associated esophageal atresia.
Surgery. 129(1):29-38, 2001
5- Vasudevan SA, Kerendi F, Lee H, Ricketts RR: Management of
congenital esophageal stenosis. J Pediatr Surg. 37(7):1024-6, 2002
6- Amae S, Nio M, Kamiyama T, Ishii T, Yoshida S, Hayashi Y, Ohi R:
Clinical characteristics and management of congenital esophageal
stenosis: a report on 14 cases. J Pediatr Surg. 38(4):565-70, 2003
Ovarian Masses
Ovarian masses can pose a problem in
diagnosis and management in children. Almost 10% of such ovarian masses
are malignant. The rate of removing normal ovarian tissue in lieu of
discovering a malignancy is still reported to be very high. Also the
role of laparoscopy asa a diagnostic/therapeutic tool in such cases is
questioned. Symptoms, age at presentation, ultrasound, CT and MRI
imaging characteristics, mass size and genetic markers results comprise
the significant factors to study used to help classify whether an
ovarian mass is benign or malignant. The greatest percent in malignancy
is found in girls aged one to eight years with a 3-fold increase in
odds. Likewise is extremely rare to find a malignant tumor in infants
less than one year of age. In terms of symptoms complaint of abdominal
mass (torsion and abdominal pain) or precocious puberty increases the
risk of malignancy. Positive HCG, alpha fetoprotein and CA-125 are
potential markers of malignancy. Negative tumor markers do not exclude
the possibility of malignancy. Imaging characteristics of solid or
heterogenous consistency and size larger than 8 cm increase the risk of
malignancy, while US evidence of normal ovarian tissue adjacent to the
mass can assist to exclude malignancy. During surgery biopsy with
frozen section can help establish a diagnosis, but it has the risk of
upstaging a potential tumor. Laparoscopy can help stratify the mass
into benign or malignant looking further deciding whether an open or
closed approach is warranted.
References:
1-Ghezzi F, Cromi A, Bergamini V, Uccella S, Siesto G, Franchi M, Bolis
P: Should adnexal mass size influence surgical approach? A series of
186 laparoscopically managed large adnexal masses. BJOG. 115(8):1020-7,
2008
2- Mayer JP, Bettolli M, Kolberg-Schwerdt A, Lempe M, Schlesinger F,
Hayek I, Schaarschmidt K: Laparoscopic approach to ovarian mass in
children and adolescents: already a standard in therapy. J Laparoendosc
Adv Surg Tech A. 19 Suppl 1:S111-5, 2009
3- Islam S, Yamout SZ, Gosche JR: Management and outcomes of ovarian
masses in children and adolescents. Am Surg. 74(11):1062-5, 2008
4- Karpelowsky JS, Hei ER, Matthews K: Laparoscopic resection of benign
ovarian tumours in children with gonadal preservation. Pediatr Surg
Int. 25(3):251-4, 2009
5- Brookfield KF, Cheung MC, Koniaris LG, Sola JE, Fischer AC: A
population-based analysis of 1037 malignant ovarian tumors in the
pediatric population. J Surg Res. 156(1):45-9, 2009
6- Stankovi Z, Bjelica A, Djuki MK, Savi D: The value of
ultrasonographic detection of normal ovarian tissue in the differential
diagnosis of adnexal masses in pediatric patients. Ultrasound Obstet
Gynecol. Jan 12, 2010
7- Oltmann SC, Garcia N, Barber R, Huang R, Hicks B, Fischer A: Can we
preoperatively risk stratify ovarian masses for malignancy? J Pediatr
Surg 45(1): 130-134, 2010
Oophoropexy
Pexing one or both ovaries is
indicated in the setting of ovarian torsion and to protect the ovaries
from further damage by adjuvant radiotherapy to nearby malignancies. In
the early 80's oophoropexy was utilized in Hodgskin's disease after
extensive radiotherapy. With the advent of favoring chemotherapy
instead of total nodal radiation the need for oophoropexy decreased
significantly. The other indication for lateral oophoropexy is when the
child is to receive craniospinal irradiation for a radiosensitive
central nervous system tumor. Laparoscopic oophoropexy may protect
against radiation-induced ovarian failure. Still, another indication
for oophoropexy is in the setting of ovarian torsion with possible or
imminent loss of ovarian tissue. The consensus is that contralateral
laparoscopic oophoropexy at the time of ipsilateral oophorectomy for
torsion is recommended to avoid castration. Medial oophoropexy to avoid
tubo-ovarian disturbance is recommended in such situation.
References:
1- Gabriel DA, Bernard SA, Lambert J, Croom RD 3rd: Oophoropexy and the
management of Hodgkin's disease. A reevaluation of the risks and
benefits. Arch Surg. 1986 Sep;121(9):1083-5
2- Shun A: Unilateral childhood ovarian loss: an indication for contralateral oophoropexy? Aust N Z J Surg. 60(10):791-4, 1990
3- Germain M, Rarick T, Robins E: Management of intermittent ovarian
torsion by laparoscopic oophoropexy. Obstet Gynecol. 88(4 Pt 2):715-7,
1996
4- Nagel TC, Sebastian J, Malo JW: Oophoropexy to prevent sequential or
recurrent torsion. J Am Assoc Gynecol Laparosc. 4(4):495-8, 1997
5- Steyaert H, Meynol F, Valla JS: Torsion of the adnexa in children:
the value of laparoscopy. Pediatr Surg Int. 13(5-6):384-7, 1998
6- Eckler K, Laufer MR, Perlman SE: Conservative management of
bilateral asynchronous adnexal torsion with necrosis in a prepubescent
girl. J Pediatr Surg. 35(8):1248-51, 2000
7- Celik A, Ergün O, Aldemir H, Ozcan C, Ozok G, Erdener
A, Balýk E: Long-term results of conservative management
of adnexal torsion in children. J Pediatr Surg. 40(4):704-8, 2005
8- Kuohung W, Ram K, Cheng DM, Marcus KJ, Diller LR, Laufer MR:
Laparoscopic oophoropexy prior to radiation for pediatric brain tumor
and subsequent ovarian function. Hum Reprod. 23(1):117-21, 2008
PSU Volume 34 No 04 APRIL 2010
Thoracoscopic Lung Biopsy
The first minimally invasive procedure
reported using thoracoscopic techniques was a lung biopsy. Lung biopsy
is generally utilized to provide answers for causes of interstitial
lung disease in both the immunocompetent and immunocompromised child,
lung residual masses after chemotherapy and evaluation of new
suspicious nodules found in oncology patients. Interstitial lung
disease is defined as diffuse interstitial findings on chest x-ray or
CT scan. Interstitial disease may be bilateral or unilateral and may be
more prominent in one lobe over another. Posterior pleural
biopsies are performed with the patient almost prone and anterior
lesions are performed with the patient almost supine. Thus, positioning
takes advantage of gravity to allow the lung to fall away from the
lesion when the lung is collapsed. Instead of single lung ventilation
children can benefit from undergoing contralateral lung ventilation
using ipsilateral bronchial blockers or Fogarty balloon catheters. Use
of CO2 insufflation creates a pneumothorax and further collapses the
ipsilateral lung. Pressures of 4-6 mm should be utilized. The lung
biopsy can be performed with endoloop or endoscopic staplers.
Image-guided percutaneous needle biopsy is preferred for pulmonary
nodules. Thoracoscopy reduces pain, shortens hospital stay and is more
pleasing cosmetically.
References:
1- Rothenberg SS, Wagner JS, Chang JH, Fan LL: The safety and efficacy
of thoracoscopic lung biopsy for diagnosis and treatment in
infants and children. J Pediatr Surg. 31(1):100-3, 1996
2- Partrick DA, Bensard DD, Teitelbaum DH, Geiger JD, Strouse P, Harned
RK: Successful thoracoscopic lung biopsy in children utilizing
preoperative CT-guided localization. J Pediatr Surg. 37(7):970-3, 2002
3-Hayes-Jordan A, Connolly B, Temple M, Chait P, Weitzman S, Njere I,
Langer JC, Kim P: Image-guided percutaneous approach is superior to the
thoracoscopic approach in the diagnosis of pulmonary nodules in
children. J Pediatr Surg. 38(5):745-8, 2003
4- Ponsky TA, Rothenberg SS: Thoracoscopic lung biopsy in infants and
children with endoloops allows smaller trocar sites and discreet
biopsies. J Laparoendosc Adv Surg Tech A. 18(1):120-2, 2008
5- Langenburg SE, Lelli JL: Minimally invasive surgery of the lung:
lung biopsy, treatment of spontaneous pneumothorax, and pulmonary
resection. Semm Pediatr Surg 17: 30-33, 2008 6-
Glüer S, Schwerk N, Reismann M, Metzelder ML, Nustede R,
Ure BM, Gappa M: Thoracoscopic biopsy in children with diffuse
parenchymal lung disease. Pediatr Pulmonol. 43(10):992-6, 2008
Thoracoscopic CDH Repair
Congenital diaphragmatic hernias (CDH)
can be surgically managed using either laparoscopic or thoracoscopic
minimally invasive technique. Either Bochdalek and Morgagni hernias
have lent themselves to repair using these minimally invasive approach.
The ideal child for thoracoscopic repair would be those that have
delayed presentation beyond the neonatal period since the presence of
pulmonary hypoplasia and pulmonary hypertension is minimal or none. The
child should be in stable cardiovascular and respiratory status before
surgery. The procedure is performed under general anesthesia without
single lung ventilation with an epidural thoracic catheter. Reduction
of the hernia occurs using one optical trocar, two operating trocars
and a pleural insufflation pressure of carbon dioxide between five and
8 mmHg. The hernia defect is repaired using non-absorbable interrupted
sutures or mesh with absence of significant diaphragm. Conversion to
open occur with difficulty in reducing the hernia toward the abdominal
cavity, herniation of liver and intolerance of insufflation.
Reduction of the defect is easier in babies that have a hernial sac.
Without a sac the bowel is gently pushed down into the abdominal
cavity. Using the laparoscopic approach the spleen and bowel are
difficult to reduce into the peritoneal cavity and the working space is
very restricted. The thoracoscopic technique causes minimal trauma,
results in good respiratory function, excellent cosmetic results and
promotes early recovery. End tidal CO2 is significantly elevated during
repair.
References:
1-Becmeur F, Jamali RR, Moog R, Keller L, Christmann D, Donato L,
Kauffmann I, Schwaab C, Carrenard G, Sauvage P: Thoracoscopic treatment
for delayed presentation of congenital diaphragmatic
hernia in the infant. A report of three cases. Surg Endosc. 15(10):1163-6, 2001
2- Nguyen TL, Le AD: Thoracoscopic repair for congenital diaphragmatic
hernia: lessons from 45 cases. J Pediatr Surg. 41(10):1713-5, 2006
3- Becmeur F, Reinberg O, Dimitriu C, Moog R, Philippe P: Thoracoscopic
repair of congenital diaphragmatic hernia in children. Semin Pediatr
Surg. 16(4):238-44, 2007
4- Guner YS, Chokshi N, Aranda A, Ochoa C, Qureshi FG, Nguyen NX,
Grikscheit T, Ford HR, Stein JE, Shin CE: Thoracoscopic repair of
neonatal diaphragmatic hernia.J Laparoendosc Adv Surg Tech A.
18(6):875-80, 2008
5- Cho SD, Krishnaswami S, Mckee JC, Zallen G, Silen ML, Bliss DW:
Analysis of 29 consecutive thoracoscopic repairs of congenital
diaphragmatic hernia in neonates compared to historical controls. J
Pediatr Surg. 44(1):80-6, 2009
6- Gourlay DM, Cassidy LD, Sato TT, Lal DR, Arca MJ: Beyond
feasibility: a comparison of newborns undergoing thoracoscopic and open
repair of congenital diaphragmatic hernias. J Pediatr Surg.
44(9):1702-7, 2009
7- McHoney M, Giacomello L, Nah SA, De Coppi P, Kiely EM, Curry JI,
Drake DP, Eaton S, Pierro A: Thoracoscopic repair of congenital
diaphragmatic hernia: intraoperative ventilation and recurrence. J
Pediatr Surg. 45(2):355-359, 2010
Anal Strictures
Anal stricture is a well known
complication of anorectal surgery in children and adults. Causes of
anal stricture includes anastomotic ischemia, anastomotic leak or
deshicence, and local infection. Anal stricture can occur after
anorectal procedures for Hisrchsrpungs disease, Familial polyposis,
inflammatory bowel disease, imperforate anus and anorectal tumors. Most
surgeons will start postoperative dilatation two weeks after surgery.
This is followed by a program of gradual dilatations until the area
becomes pliable, soft and symptoms disappear. Some strictures are not
amenable to dilatation and will need further surgery by anoplasty or
repeated pull-through. Mitomycin C, an agent that inhibits RNA and
protein synthesis has been shown to be effective in the prevention of
fibroblast proliferation that leads to scarring, has been utilized in
esophageal, vaginal, laryngeal and urethral strictures with success.
Mitomycin C application has recently been utilized in anal strictures
as an adjunct to anorectal dilatations with success avoiding the need
of further reconstructive surgery.
References:
1-Liberman H, Thorson AG: How I do it. Anal stenosis. Am J Surg. 179(4):325-9, 2000
2- Alexander F: Complications of ileal pouch anal anastomosis. Semin Pediatr Surg. 16(3):200-4, 2007
3- Daher P, Riachy E, Georges B, Georges D, Adib M: Topical application
of mitomycin C in the treatment of esophageal and tracheobronchial
stricture: a report of 2 cases. J Pediatr Surg. 42(9):E9-11, 2007
4- Heran MK, Baird R, Blair GK, Skarsgard ED: Topical mitomycin-C for
recalcitrant esophageal strictures: a novel endoscopic/fluoroscopic
technique for safe endoluminal delivery. J Pediatr Surg. 43(5):815-8,
2008
5- Lillehei CW, Leichtner A, Bousvaros A, Shamberger RC: Restorative
proctocolectomy and ileal pouch-anal anastomosis in children. Dis Colon
Rectum. 52(9):1645-9, 2009
6- Mueller CM, Beaunoyer M, St-Vil D: Topical mitomycin-C for the treatment of anal stricture. J Pediatr Surg. 45(1):241-4, 2010
PSU Volume 34 No 05 MAY 2010
Paraganglioma
Paraganglioma is a neural
crest-derived catecholamine-secreting cell tumor originating in
extraadrenal, usually paravertebral location. Its twin brother is
the pheochromocytoma, which arises from the adrenal medulla. These
tumors can occur at any age, though most arise during the third through
the fifth decade of life. Children are more prone to develop
paragangliomas. In children the head and neck are primarily affected
followed by the retroperitoneum presenting as a palpable mass or pain.
Paragangliomas can be associated with MEN2A, MEN2B and von
Hippel-Lindau disease, though most cases in children are sporadic. From
a diagnostic medical perspective is usually unimportant whether the
tumor is a pheochromocytoma or a paraganglioma. Surgically this
distinction is important since the approach may be dictated by the
preoperative imaging studies. Excess catecholamine secretion
occasionally occurs during intraoperative manipulation. Paragangliomas
are classified as malignant when distant metastasis are present, the
tumor is unresectable, or the tumor recurs regionally or distantly
after initial resection with tumor negative margins. Complete surgical
resection during initial presentation is the treatment of choice for
paragangliomas and the only factor related to a favorable outcome.
Neoadjuvant chemotherapy, radiotherapy and high dose of iodine 131-MIBG
therapy is reserved for patients with features suggestive of incomplete
resection or malignancy. Angioembolization is a last resort technique
for unresectable symptomatic tumors.
References:
1- Tekautz TM, Pratt CB, Jenkins JJ, Spunt SL: Pediatric extraadrenal paraganglioma. J Pediatr Surg. 38(9):1317-21, 2003
2- Kravarusic D, Pinto-Rojas A, Al-Assiri A, Sigalet D: Laparoscopic
resection of extra-adrenal pheochromocytoma--case report and review of
the literature in pediatric patients. J Pediatr Surg. 42(10):1780-4,
2007
3- Rabah R, Weber R, Serhatkulu GK, Cao A, Dai H, Pandya A, Naik R,
Auner G, Poulik J, Klein M: Diagnosis of neuroblastoma and
ganglioneuroma using Raman spectroscopy. J Pediatr Surg.
43(1):171-6, 2008
4- Bissada NK, Safwat AS, Seyam RM, Al Sobhi S, Hanash KA, Jackson RJ,
Sakati N, Bissada MA: Pheochromocytoma in children and adolescents: a
clinical spectrum. J Pediatr Surg. 43(3):540-3, 2008
5- Krauel L, Albert A, Mora J, Sola T, Cruz O, Mortera C,
Ribó JM: Use of angioembolization as an effective
technique for the management of pediatric solid tumors. J Pediatr
Surg. 44(9):1848-55, 2009
6- Hammond PJ, Murphy D, Carachi R, Fraser D, McIntosh D: Childhood
phaeochromocytoma and paraganlioma: 100% incidence of genetic mutations
and 100% survival. J Pediatr Surg 45(2): 383-386, 2010
Lung Abscess
Lung abscess occurs rarely in the
pediatric population. It is defined as a thick-walled cavity of
localized infection with an area of central necrosis and suppuration
within the lung parenchyma. They can lead to cavitation, necrosis and
destruction of such lung parenchyma. Most children present with fever,
cough and dyspnea. Lung abscess are classified as primary if they are
solitary and occurring in a healthy patient after a community-acquired
pneumonia. Secondary lung abscess are usually multiple, right-sided and
occurs in children with another medical condition such as
immunodeficiency, immunosuppression, recurrent aspiration or cystic
fibrosis. Lung abscess results from both aerobic and anaerobic
bacterias with almost one-third of them mixed organisms. S Pneumoniae
and S Aureus are the most common offending organisms. Beside simple
chest films, diagnostic imaging should included a chest ultrasound. CT
Scan is reserve when ultrasound is technically limited or discrepant in
findings. Management of lung abscess is long-term (at least six weeks)
broad-spectrum antibiotic therapy. If medical therapy fails or a
bronchopleural fistula develops invasive intervention is needed. This
might consist of percutaneous drainage under CT guidance, bronchoscopic
drainage, formal lobectomy, wedge resection or thoracoscopic drainage.
Thoracoscopic drainage has lead to quick resolution of the abscess with
little morbidity and nil long-term bronchopleural fistula
formation.
References:
1- Wu MH, Tseng YL, Lin MY, Lai WW: Surgical treatment of pediatric lung abscess. Pediatr Surg Int. 12(4):293-5, 1997
2- Cowles RA, Lelli JL Jr, Takayasu J, Coran AG: Lung resection in
infants and children with pulmonary infections refractory to medical
therapy. J Pediatr Surg. 37(4):643-7, 2002
3-Chan PC, Huang LM, Wu PS, Chang PY, Yang TT, Lu CY, Lee PI, Chen JM,
Lee CY, Chang LY: Clinical management and outcome of childhood lung
abscess: a 16-year experience. J Microbiol Immunol Infect. 38(3):183-8,
2005
4- Puligandla PS, Laberge JM: Respiratory infections: pneumonia, lung
abscess, and empyema. Semin Pediatr Surg. 17(1):42-52, 2008
5- Kurian J, Levin TL, Han BK, Taragin BH, Weinstein S: Comparison of
ultrasound and CT in the evaluation of pneumonia complicated by
parapneumonic effusion in children. AJR Am J Roentgenol.
193(6):1648-54, 2009
6- Nagasawa KK, Johnson SM: Thoracoscopic treatment of pediatric lung abscesses. J Pediatr Surg. 45(3):574-578, 2010
Fecal Impaction
Impaction of fecal material is a very
common problem in children, the result of chronic constipation. Every
child with chronic constipation since birth should be evaluated for
rectal biopsy, since aganglionosis is a surgical correctable cause of
constipation. Constipation with fecal impaction in children usually is
functional and the result of stool retention. Fecal impaction can cause
fever, leukocytosis, general malaise, vomiting, bowel obstruction,
rectal bleeding, abdominal pain and distension with failure to thrive.
In its severe form the child can develop toxic megacolon, respiratory
arrest and colon perforation. Diagnosis begins with recognition of
possible fecal impaction and confirmation by digital examination or
abdominal radiography. Management of idiopathic fecal impaction
consists of manual disimpaction, isotonic oral lavage (PEG) and saline
enemas. After disimpaction, a maintenance program may be required for
months to years because relapse of functional constipation is common.
Maintenance medications include mineral oil, lactulose, milk of
magnesia, polyethylene glycol powder, and sorbitol.
References:
1- Youssef NN, Peters JM, Henderson W, Shultz-Peters S, Lockhart DK, Di
Lorenzo C: Dose response of PEG 3350 for the treatment of childhood
fecal impaction. J Pediatr. 141(3):410-4, 2002
2- Biggs WS, Dery WH: Evaluation and treatment of constipation in infants and children.Am Fam Physician. 73(3):469-77, 2006
3- Tobias N, Mason D, Lutkenhoff M, Stoops M, Ferguson D: Management
principles of organic causes of childhood constipation. J Pediatr
Health Care. 22(1):12-23, 2008
4- Wald A: Management and prevention of fecal impaction.Curr Gastroenterol Rep. 10(5):499-501, 2008
5- Candy D, Belsey J: Macrogol (polyethylene glycol) laxatives in children with functional constipation
and faecal impaction: a systematic review. Arch Dis Child. 94(2):156-60, 2009
6- Bekkali NL, van den Berg MM, Dijkgraaf MG, van Wijk MP, Bongers ME,
Liem O, Benninga MA: Rectal fecal impaction treatment in childhood
constipation: enemas versus high doses oral PEG. Pediatrics.
124(6):e1108-15, 2009
PSU Volume 34 No 06 JUNE 2010
H-type Rectovestibular Fistula
H-type rectovestibular fistulas, also
called congenital rectovestibular fistula with a normal anus, is a rare
condition encountered in Western female infants, except in Asia where
is encountered more commonly. Clinically the anus is in a normal
position while the child discharges fecal material or flatus from the
vestibule or vagina. Most of these fistulas extend from the posterior
vestibular fourchette to the anterior wall of the rectum located below
the level of the levator muscles and one to three cm above the dentate
line. No patient has history of perineal abscess or cellulitis in the
congenital variety. Some cases are associated with a stenotic or
anteriorly displace anal canal. Müllerian duct structure agenesis
(Mayer-Rokitansky syndrome) has been associated with this type of
fistula. The fistula is lined by squamous epithelium and most have a
small diameter (1-5 mm). Management consists of resection of the
fistulous tract preferably at the age of three years when the perineal
body is better developed. Several procedures have been reported in the
literature such as simple fistula resection, the vestibuloanal
pull-through, transperineal closure of the fistula, posterior sagittal
anorectal approach and a transanal approach. Most of the procedures can
be performed without the need of a protective colostomy, but a good
bowel preparation is needed. Complications include wound infection and
fistula recurrence with reoperation in 50% of the cases.
References:
1- Rintala RJ, Mildh L, Lindahl H: H-type anorectal malformations:
incidence and clinical characteristics. J Pediatr Surg. 31(4):559-62,
1996
2- Willems M, Kluth D, Lambrecht W: Anorectal malformation: a new
anatomic variant resembling an H-type fistula. J Pediatr Surg.
31(12):1682-4, 1996
3- Kulshrestha S, Gangopadhyay AN, Sahoo SP: An unusual variant of
rectal atresia with rectovestibular fistula.J Pediatr Surg.
32(6):921-2, 1997
4- Tsugawa C, Nishijima E, Muraji T, Satoh S, Kimura K: Surgical repair
of rectovestibular fistula with normal anus. J Pediatr Surg.
34(11):1703-5, 1999
5- Mahajan JK, Venkatesh MA, Bawa M, Rao KL:
Mayer-Rokitansky-Kuster-Hauser syndrome with H-type anovestibular
fistula. J Pediatr Surg. 44(8):E1-3, 2009
6- Li L, Zhang TC, Zhou CB, Pang WB, Chen YJ, Zhang JZ: Rectovestibular
fistula with normal anus: a simple resection or an extensive perineal
dissection? J Pediatr Surg. 45(3):519-24, 2010
Rhabdoid Tumor
Rhabdoid tumor is a rare, very
aggressive malignancy identified in fetus and young children. Rhabdoid
tumors (RT) can occur within the kidney, central nervous system and
extrarenal in location. Rhabdoid tumors are characterized by early
metastasis and high mortality rate. Most cases are identified in the
perinatal period during the first year of life. The tumor metastasize
to multiple sites such as skin, placenta, bones, lungs, lymph nodes,
brain and liver. Extrarenal RT occurs more commonly in the perinatal
period, presents as a mass lesion in head/neck, skin tumor nodules or
metastatic disease, has a bad prognosis. Renal RT occurs primarily in
infants and newborns, presents as an abdominal mass, fever and
hematuria, with high-tumor stage. Most have metastasis at diagnosis
with presence of CNS lesions. Survival increases with increasing age at
diagnosis. Central nervous system RT occurs primarily in infants in the
posterior fossa presenting as an intracranial mass and hydrocephalus,
characterized by extensive brain invasion, recurrence and short
survival. For cure an aggressive surgical approach to achieve total
gross resection of the tumor is needed along with adjuvant chemo- and
radiotherapy. Patients with localized disease and complete surgical
resection are most likely to survive long-term.
References:
1- Amar AM, Tomlinson G, Green DM, Breslow NE, de Alarcon PA: Clinical
presentation of rhabdoid tumors of the kidney. J Pediatr Hematol Oncol.
23(2):105-8, 2001
2- Tomlinson GE, Breslow NE, Dome J, Guthrie KA, Norkool P, Li S,
Thomas PR, Perlman E, Beckwith JB, D'Angio GJ, Green DM: Rhabdoid tumor
of the kidney in the National Wilms' Tumor Study: age at diagnosis as a
prognostic factor. J Clin Oncol. 20;23(30):7641-5, 2005
3-Madigan CE, Armenian SH, Malogolowkin MH, Mascarenhas L: Extracranial
malignant rhabdoid tumors in childhood: the Childrens Hospital Los
Angeles experience. Cancer. 110(9):2061-6, 2007
4- Reinhard H, Reinert J, Beier R, et al: Rhabdoid tumors in children:
prognostic factors in 70 patients diagnosed in Germany. Oncol Rep.
19(3):819-23, 2008
5- Wu X, Dagar V, Algar E, Muscat A, Bandopadhayay P, Ashley D, Wo Chow
C: Rhabdoid tumour: a malignancy of early childhood with variable
primary site, histology and clinical behaviour. Pathology.
40(7):664-70, 2008
6- Sultan I, Qaddoumi I, Rodriguez-Galindo C, Nassan AA, Ghandour K,
Al-Hussaini M: Age, stage, and radiotherapy, but not primary tumor
site, affects the outcome of patients with malignant rhabdoid tumors.
Pediatr Blood Cancer. 54(1):35-40, 2010
7- Isaacs H: Fetal and neonatal rhabdoid tumor. J Pediatr Surg 45 (3):619-626, 2010
Thoracic Duct Ligation
The lymphatics of the lower extremity
and lower torso join with those of the mesentery of the bowel to form
the cisterna chyli. The cisterna chyli lies in the lumbar prevertebral
plane behind the aorta and inferior vena cava. Confluents of the
cisterna chyli create the thoracic duct which ascends in the right
thoracic prevertebral plane, medial to the azygos vein and behind the
esophagus and aorta. The thoracic duct is a tubular structure one to 3
mm in diameter. It crosses from the right side of the chest to the left
at the level of the fourth or fifth thoracic vertebrae and it usually
empties into the left jugulo-subclavian junction. The accessory
thoracic duct empties in a similar fashion on the right side. Either
contrast or scintigraphic lymphangiography can help delineate the
anatomic variants identified in almost 40% of human. Direct contrast
lymphangiography is considered the best technique to delineate the
anatomy of aberrant lymphatics channels. Postsurgical, traumatic,
congenital or spontaneous chylothorax that does not respond to medical
management with drainage, medium-chain triglyceride, or TPN might need
ligation of the thoracic duct. Low thoracic duct ligation is a reliable
means of control of postoperative chylothorax. Ligation can be
performed through either right chest thoracotomy or video-assisted
thoracoscopy.
References:
1- Johnson DC, Cartmill TB: Low thoracic duct ligation for
postoperative chylous effusions in infants and children. Aust N Z J
Surg. 47(1):94-9, 1977
2- Milsom JW, Kron IL, Rheuban KS, Rodgers BM: Chylothorax: an
assessment of current surgical management. J Thorac Cardiovasc Surg.
89(2):221-7, 1985
3- Stringel G, Teixeira JA: Thoracoscopic ligation of the thoracic duct. JSLS. 4(3):239-42, 2000
4- Fishman SJ, Burrows PE, Upton J, Hendren WH: Life-threatening
anomalies of the thoracic duct: anatomic delineation dictates
management. J Pediatr Surg. 36(8):1269-72, 2001
5- Achildi O, Smith BP, Grewal H: Thoracoscopic ligation of the
thoracic duct in a child with spontaneous chylothorax. J
Laparoendosc Adv Surg Tech A. 16(5):546-9, 2006
6- Soto-Martinez M, Massie J: Chylothorax: diagnosis and management in children. Paediatr Respir Rev. 10(4):199-207, 2009