PEDIATRIC SURGERY UPDATE ©
VOLUME 32, 2009
PSU Volume 32 No 01 JANUARY 2009
Thyroid Nodules
Management of thyroid nodules in children should proceed well ordered
since deviations in algorithm causes significant delay in diagnosis, morbidity
and mortality in the face of a disease that is curable most of the time.
Initially a child with a thyroid nodule should undergo blood sampling for
T3, T4, TSH, thyroglobulin along with simple chest films to determine if
he is euthyroid, hypo- or hyperthyroid. First imaging study that should
be done is a neck ultrasound to determine if the nodule is solid, cystic
or complex. If the child harbors a solid mass and is hyperthyroid a thyroid
scan will help determine if the cause is an autonomous nodule or a completely
enhancing gland. Otherwise next step in management is performing a fine
needle aspiration (FNA) cytology. If the cyst disappears with aspiration
a period of watchful follow-up is justified. The FNA will help determine
of the child has a benign, probable or unmistaken malignant lesion. With
a persistent benign lesions a period of watchful waiting or hemithyroidectomy
if the lesion persists or grow is justified. A probable malignant
lesion can be managed with hemithyroidectomy and completion thyroidectomy
if the pathology comes malignant. An unmistaken malignant lesion should
undergo total thyroidectomy with central node compartment dissection. With
multicentricity, lymph node involvement, persistently high thyroglobulin
levels, or distant metastasis ablation radioiodine is required.
References:
1- Lugo-Vicente HL, Ortíz V, Irizarry H, Camps
JI, Pagán V : Pediatric Thyroid Nodules: Management in the Era of
FNA: J Pediatr Surg 33(8):1302-1305, 1998
2- Spinelli C, Bertocchini A, Antonelli A, Miccoli P:
Surgical therapy of the thyroid papillary carcinoma in children: experience
with 56 patients < or =16 years old. J Pediatr Surg. 39(10):1500-5,
2004
3- Shapiro NL, Bhattacharyya N: Population-based outcomes
for pediatric thyroid carcinoma.Laryngoscope. 115(2):337-40, 2005
4- Palmer BA, Zarroug AE, Poley RN, Kollars JP, Moir
CR: Papillary thyroid carcinoma in children: risk factors and complications
of disease recurrence. J Pediatr Surg. 40(8):1284-8, 2005
5- Savio R, Gosnell J, Palazzo FF, Sywak M, Agarwal G,
Cowell C, Shun A, Robinson B, Delbridge LW: The role of a more extensive
surgical approach in the initial multimodality management of papillary
thyroid cancer in children. J Pediatr Surg. 40(11):1696-700, 2005
6- Canadian Pediatric Thyroid Nodule (CaPTN) Study Group:
The Canadian Pediatric Thyroid Nodule Study: an evaluation of current management
practices. J Pediatr Surg. 43(5):826-30, 2008
Stealth Surgery
Stealth surgery refers to endoscopic subcutaneous procedures performed
relatively invasive without leaving any obvious evidence that an operation
has occurred. The concept refers to excision of benign subcutaneous lesions
of the head and neck in children using an incision in the axilla or through
hidden incision in the scalp. Most of these subcutaneous lesions are approached
directly on top of them usually using cosmetic skin creases to avoid an
unsightly scar. In some cases the scarring could be displeasing to patient
and family. Lesions include dermoid cysts, lymph node biopsy, thyroglossal
duct cysts, ectopic dilated veins, small hemangiomas, cutting of the sternocleidomastoid
muscle for torticollis, benign thyroid lobectomy and removal of parathyroid
adenomas. The technique uses a laparoscope and two other trocars place
near the axilla away from the lesion. The subcutaneous space is insufflated
with carbon dioxide to expand and create a working space the same as is
done during abdominal laparoscopic procedures. Procedures can be performed
ambulatory. Beside excellent cosmetic results other advantages include
magnified visualization of the anatomy and the lesions. Children whose
lesions are suspected to be malignant, lymphangiomas, hemangiomas or involving
the skin are excluded.
References:
1- Dutta S, Lorenz HP, Albanese CT: Endoscopic excision
of benign forehead masses: a novel approach for pediatric general surgeons.
J Pediatr Surg. 41(11):1874-8, 2006
2- Swain B: Transaxillary endoscopic release of restricting
bands in congenital muscular torticollis--a novel technique. J Plast Reconstr
Aesthet Surg. 60(1):95-8, 2007
3- Miyano G, Lobe TE, Wright SK: Bilateral transaxillary
endoscopic total thyroidectomy. J Pediatr Surg. 43(2):299-303, 2008
4- Dutta S, Albanese CT: Transaxillary subcutaneous endoscopic
release of the sternocleidomastoid muscle for treatment of persistent torticollis.
J Pediatr Surg. 43(3):447-50, 2008
5- Dutta S, Slater B, Butler M, Albanese CT: "Stealth
Surgery": transaxillary subcutaneous endoscopic excision of benign neck
lesions. J Pediatr Surg 43(11): 2070-2074, 2008
Cystic Nephroma
Cystic nephroma (CF) is a very rare benign cystic renal neoplasm seen
in both children and adults characterized by a solitary, well-circumscribed,
multiseptate mass of non-communicating, fluid-filled loculi surrounded
by a thick fibrous capsule compressing normal renal parenchyma. Etiology
is unknown. CF is seen in patients older than 30 years with a male to female
ratio of 1:8. The cyst can involve partially or completely the kidney,
though CF is usually unilateral and occurs sporadically. The cysts may
prolapse toward the renal pelvis causing urinary obstruction. Clinical
signs include abdominal mass, abdominal or flank pain, hematuria, hypertension
and urinary tract infection. The CT Scan is diagnostic showing a homogenous
multiloculated cystic mass with capsule resembling a football. Differential
diagnosis includes cystic Wilms tumor, multicystic renal dysplasia, cystic
hamartoma and congenital mesoblastic nephroma. Operative intervention is
indicated in all of these cases to establish diagnosis and procure treatment.
Goals of surgery consist of eradication of all tumor tissue with preservation
of as much renal tissue as possible. This can be accomplished with enucleation
or partial nephrectomy. Histologic diagnosis is imperative before any adjuvant
therapy is started. The localized form, when asymptomatic, can be managed
more conservatively.
References:
1- Sacher P, Willi UV, Niggli F, Stallmach T: Cystic
nephroma: a rare benign renal tumor. Pediatr Surg Int. 13(2-3):197-9, 1998
2- Rebassa Llull MJ, Muñoz Vélez D, Hidalgo
Pardo F, Gutiérrez Sanz-Gadea C, Mus Malleu A, Torrens Darder I,
Antón Valentí E, Ozonas Moragues M: Cystic nephroma. Report
of 5 cases. Arch Esp Urol. 53(5):476-9, 2000
3- Boulanger SC, Brisseau GF: Cystic nephroma: a benign
renal tumor of children and adults. Surgery. 133(5):596-7, 2003
4- Bouhafs A, Cherradi N, Lamaalmi N, Belkacem R, Barahioui
M: An unusual case of multilocular cystic nephroma with prominent renal
pelvis involvement. Int J Urol. 13(4):436-8, 2006
5- Luithle T, Szavay P, Furtwängler R, Graf N, Fuchs
J; SIOP/GPOH Study Group: Treatment of cystic nephroma and cystic partially
differentiated nephroblastoma--a report from the SIOP/GPOH study group.
J Urol. 177(1):294-6, 2007
6- Boybeyi O, Karnak I, Orhan D, Ciftci AO, Tanyel FC,
Kale G, Senocak ME: Cystic nephroma and localized renal cystic disease
in children: diagnostic clues and management. J Pediatr Surg. 43(11):1985-9,
2008
PSU Volume 32 No 02 FEBRUARY 2009
RadioiodineTherapy for Thyroid Cancer
The thyroid gland is very efficient in trapping iodine. Radioiodine
therapy in the form of Iodine-131 is utilized in the adjunctive management
of well-differentiated thyroid malignancy such as papillary and follicular
carcinoma. Radioiodine therapy (RxT) is used to (1) ablate residual normal
thyroid tissue after subtotal thyroidectomy or lobectomy, and (2) manage
functioning metastases from thyroid cancer. Metastases can occur to the
regional nodes, lung, bone and liver. After total thyroidectomy, plasma
thyroglobulin is the most useful marker for monitoring tumor progression.
Residual thyroid ablation can occur with a single dose of I-131. In the
setting of metastatic disease RxT is most effective 6-8 weeks after total
thyroidectomy when the TSH is above 35 mIU/mL and the thyroid tissue within
the metastatic foci is very avid in taking iodine. Dosimetry studies are
done to determine the safe maximal dose of iodine-131 to be administered
to the child. Depending on the site of metastasis (lymph nodes, lung or
bone) will depend the quantity of radiotracer to be given (approximately
150-175, 175-200, or 200 mCi respectively).Side effects of RxT takes years
to develop. After therapy the child can have nausea, emesis, transitory
& reversible thrombocytopenia and sialadenitis. Development of second
tumors is very rare. I-131 may cause impairment of testicular function.
There is no evidence that exposure to radioiodine affects the outcomes
of subsequent pregnancies and offspring. Long term prognosis is excellent.
References:
1- Yeh SDJ, La Quaglia MP: 131-I Therapy for Pediatric
Thyroid Cancer: Semm Pediatr Surg 6(3): 128-133, 1997
2- La Quaglia MP, Black T, Holcomb GW 3rd, Sklar C, Azizkhan
RG, Haase GM, Newman KD: Differentiated thyroid cancer: clinical characteristics,
treatment, and outcome in patients under 21 years of age who present with
distant metastases. A report from the Surgical Discipline Committee of
the Children's Cancer Group. J Pediatr Surg. 35(6):955-9, 2000
3- Jarzab B, Handkiewicz-Junak D, Wloch J: Juvenile differentiated
thyroid carcinoma and the role of radioiodine in its treatment: a qualitative
review. Endocr Relat Cancer. 12(4):773-803, 2005
4- Durante C, Haddy N, Baudin E, Leboulleux S, Hartl
D, Travagli JP, Caillou B, Ricard M, Lumbroso JD, De Vathaire F, Schlumberger
M: Long-term outcome of 444 patients with distant metastases from papillary
and follicular thyroid carcinoma: benefits and limits of radioiodine therapy.
J Clin Endocrinol Metab. 91(8):2892-9, 2006
5- Rosário PW, Barroso AL, Rezende LL, Padrão
EL, Borges MA, Guimarães VC, Purisch S: Testicular function after
radioiodine therapy in patients with thyroid cancer. Thyroid. 16(7):667-70,
2006
6- Handkiewicz-Junak D, Wloch J, Roskosz J, Krajewska
J, Kropinska A, Pomorski L, Kukulska A, Prokurat A, Wygoda Z, Jarzab B:
Total thyroidectomy and adjuvant radioiodine treatment independently decrease
locoregional recurrence risk in childhood and adolescent differentiated
thyroid cancer. J Nucl Med. 48(6):879-88, 2007
7- Garsi JP, Schlumberger M, Rubino C, Ricard M, Labbé
M, Ceccarelli C, Schvartz C, Henri-Amar M, Bardet S, de Vathaire F: Therapeutic
administration of 131I for differentiated thyroid cancer: radiation dose
to ovaries and outcome of pregnancies. J Nucl Med. 49(5):845-52, 2008
Congenital Megalourethra
Congenital megalourethra is a very condition analogous to the extreme
form of urethral diverticulum. The megalourethra is a non-obstructive dilatation
which occurs secondary to a partial or complete agenesis of spongy and
erectile tissue. Three types are recognized: 1) localized absence of corpus
spongiosum in the penile urethra predisposing to saccular diverticulum
formation, 2) scaphoid megalourethra which is associated with a greater
deformity and deficiency of erectile tissue, and 3) the fusiform type which
is a severe deficiency of erectile tissue with almost complete absence
of corpus spongiosum and corpora cavernosa. Embryologically, megalourethra
occurs due to a failure of the mesodermal urethral folds and mesenchyme
to differentiate adequately or completely into erectile tissue. Due to
lack of adequate support the urethra balloons. Diagnosis is made upon inspection.
Multiple other anomalies might be present such as imperforate anus, cryptorchidism,
renal agenesis and aberrant adrenal tissue. Hydroureteronephrosis, megacystis
and proximal urethral dilation may be demonstrable on urographic studies.
Management consists of surgical reduction of the redundant dilated ventral
urethra for the scaphoid variety (Nesbitt urethroplasty). Preliminary urinary
diversion followed by planned staged reconstruction is necessary for the
more dreadful fusiform type.
References:
1- Wakhlu AK, Wakhlu A, Tandon RK, Kureel SN: Congenital
megalourethra. J Pediatr Surg. 31(3):441-3, 1996
2- Sharma AK, Shekhawat NS, Agarwal R, Upadhyay A, Mendoza
WX, Harjai MM: Megalourethra: a report of four cases and review of the
literature. Pediatr Surg Int. 12(5-6):458-60, 1997
3- Savanelli A, Schiano A, Esposito C, Russo S, Dolezalova
H: Congenital megalourethra associated with urethral duplication and imperforate
anus. Pediatr Surg Int. 13(8):607-9, 1998
4- Ozokutan BH, Küçükaydin M, Ceylan
H, Gözüküçük A, Karaca F: Congenital scaphoid
megalourethra: report of two cases. Int J Urol. 12(4):419-2, 2005
5- Vaghefi H, Simmons MN, Hsia MH, Ross JH: Two extremes
of the megalourethra spectrum. Urology. 67(3):614-6, 2006
6- Vallasciani S, Atzori P, Martini L, Ferro F: Scafoid
megalourethra--a reliable surgical approach. J Pediatr Surg. 43(11):2128-30,
2008
Amyand's Hernia
Amyand's hernia refers to a very rare hernia where an incarcerated or
perforated appendix is found in the right inguinal canal. It is estimated
to occur in 0.1% of all cases of appendicitis. Clinically the child presents
with a tender, nonreducible inguinal or inguinal-scrotal lump, more commonly
in the right canal than the left often imitating an incarcerated or strangulated
inguinal hernia. Symptoms may also mimic inguinal lymphadenitis, epididymis-orchitis,
hydrocele of the spermatic cord and testicular torsion. Due to the unusual
presentation the diagnosis is rarely made before surgery. Children complain
of crampy low abdominal pain combined with irreducible tender mass in the
inguinal region. Neonates can develop associated testicular ischemia. It
is believed the appendix enters into the hernia sac and its blood supply
is compromised resulting in inflammation. CT and US can be helpful in providing
a preoperative diagnosis. Management consists of repair of the hernia defect
along with appendectomy. Laparoscopy can assist the surgeon during the
appendectomy. Incidental appendicectomy in the case of a normal appendix
is not favored.
References:
1- Yazici M, Etensel B, Gürsoy H, Ozkisacik S, Erkus
M, Aydin ON: Infantile Amyand's hernia. Pediatr Int. 45(5):595-6, 2003
2- Milburn JA, Youngson GG: Amyand's hernia presenting
as neonatal testicular ischaemia. Pediatr Surg Int. 22(4):390-2, 2006
3- Sharma H, Gupta A, Shekhawat NS, Memon B, Memon MA:
Amyand's hernia: a report of 18 consecutive patients over a 15-year period.
Hernia. 11(1):31-5, 2007
4- Livaditi E, Mavridis G, Christopoulos-Geroulanos G:
Amyand's hernia in premature neonates: report of two cases. Hernia. 11(6):547-9,
2007
5- Baldassarre E, Centonze A, Mazzei A, Rubino R: Amyand's
hernia in premature twins. Hernia. 2008 Sep 13
6- Tycast JF, Kumpf AL, Schwartz TL, Coln CE: Amyand's
hernia: a case report describing laparoscopic repair in a pediatric patient.
J Pediatr Surg. 43(11):2112-4, 2008
PSU Volume 32 No 03 MARCH 2009
Congenital Diaphragmatic Hernia: Permacol
When
the defect of congenital diaphragmatic hernia (CDH) is too large to be
closed primarily, a synthetic patch must be used. This can occur with
defects larger than 50% or total agenesis of the hemidiaphragm. The
most common problem after mesh/patch repair of CDH is the high
incidence of recurrence due to poor tissue incorporation and growth
accommodation. Collagen-based bioprosthetic patches when compared with
synthetic materials demonstrate better integration with tissue and less
inflammatory response. Permacol is a sheet of collagen derived from
porcine dermis producing chemical cross-linking, making it more
resistance to collagenase degradation while retaining good tissue
integration due to the reduced inflammatory response. The cross linking
of lysine and hydroxylysine residues within the collagen fibers of
Permacol imparts a higher resistance to collagenase improving
durability. Permacol becomes incorporated by tissue ingrowth and
neovascularization. Permacol has been utilized in the adult population
for difficult abdominal wall closure in the presence of contamination,
fistula or abdominal compartment syndrome. It has also been used in the
patch repair of ileoanal pouch-vaginal fistulas. Permacol appears to be
a safe, durable alternative to synthetic patches in the closure of
large CDH defects.
References:
1- Parker DM, Armstrong PJ, Frizzi JD, North JH Jr: Porcine
dermal collagen (Permacol) for abdominal wall reconstruction. Curr
Surg. 63(4):255-8, 2006
2- Catena F, Ansaloni L, Gazzotti F, Gagliardi S, Di Saverio S,
D'Alessandro L, Pinna AD: Use of porcine dermal collagen graft
(Permacol) for hernia repair in contaminated fields. Hernia.
11(1):57-60, 2007
3- Smith M, Hooks VH, Jenkins B: Patch repair of ileoanal
pouch-vaginal fistula with Permacol collagen implant. Am Surg.
73(5):514-5, 2007
4- Gaertner WB, Bonsack ME, Delaney JP: Experimental evaluation
of four biologic prostheses for ventral hernia repair. J Gastrointest
Surg. 11(10):1275-85, 2007
5- Mitchell IC, Garcia NM, Barber R, Ahmad N, Hicks BA, Fischer
AC: Permacol: a potential biologic patch alternative in congenital
diaphragmatic hernia repair. J Pediatr Surg 43(12): 2161-2164, 2008
PET/CT: Pediatric Abdominal Tumors
Managing
children with malignancies is costly but very rewarding experience.
Positron emission tomography (PET) utilizes F-18 fluorodeoxyglucose
(FDG), a glucose analogue, that concentrates in areas of active
metabolic activities as the main radiopharmaceutical to create a PET
functional image. The FDG avidity has been demonstrated in all
abdominal tumors making it a very sensitive diagnostic modality.
Combined with a CT Scan advantage of precise anatomical detail in
evaluation of pediatric solid tumors, the PET-CT allows a combination
of functional assessment along with fine anatomical details. The PED/CT
has a sensitivity above 90%. The advantages of using a PET/CT Scan in
children with pediatric abdominal tumor malignancies include: 1)
useful in preoperative staging of the tumor and selection of
appropriate site for biopsy, 2) useful identifying occult or
unsuspected local or distant metastasis, 3) useful for follow-up of
recurrent or residual disease, especially lymphoma, 4) provides
assessment of response to adjuvant chemotherapy, and 5) valuable
where standard diagnostic studies are equivocal or conflicting. The CT
role in PET/CT is noticed when normal FDG avidity tissues such as
adenoids, thymus, thyroid, bone marrow, growth plate, brain,
myocardium, renal pelvis and bladder are evaluated. Reactive
lymphadenopathy and postop inflammation can cause false positive
studies. Irrespective of cost we need to incorporate the PET/CT into
our diagnostic armamentarium with dealing with pediatric malignancies.
References:
1- Hernandez-Pampaloni M, Takalkar A, Yu JQ, Zhuang H, Alavi A:
F-18 FDG-PET imaging and correlation with CT in staging and follow-up
of pediatric lymphomas. Pediatr Radiol. 36(6):524-31, 2006
2- Beker DB, Berrak SG, Canpolat C, Tugtepe H, Ones T, Tecimer
T: False positivity of FDG-PET/CT in a child with Hodgkin disease.
Pediatr Blood Cancer. 50(4):881-3, 2008
3- Jadvar H, Connolly LP, Fahey FH, Shulkin BL: PET and PET/CT in pediatric oncology. Semin Nucl Med. 37(5):316-31, 2007
4- Federman N, Feig SA: PET/CT in evaluating pediatric malignancies: a clinician's perspective. J Nucl Med. 48(12):1920-2, 2007
5- Servaes S, Epelman M, Pollock A, Shekdar K: Pediatric
malignancies: synopsis of current imaging techniques. Cancer Treat Res.
143:469-91, 2008
6- Murphy JJ, Tawfeeq M, Chang B, Nadel H: Early experience with
PET/CT scan in the evaluation of pediatric abdominal neoplasms. J
Pediatr Surg. 43(12):2186-92, 2008
Posttransplant Lymphoproliferative Disease
With
the increased advent of organ transplantation and immunosuppression in
children, a serious complication with a high morbidity and
mortality has raised, namely posttransplant lymphoproliferative disease
(PTLD). PTLD occurs due to abnormal lymphoid proliferation from
ineffective B-cell or T-cell function in immunosupressed patients after
solid organ transplantation. Most cases of PTLD are associated with
Epstein-Barr virus (EBV) infection. Less likely cytomegalovirus and
herpes might be involved. Incidence of developing PTLD is low for renal
transplants and higher for lung transplants. Transplant children are
more commonly affected than their adult counterpart. PTLD arises where
lymphoid tissue is present, mostly affecting head (adenoids), neck,
mediastinum and abdomen. Children present with fever, weight loss,
lethargy, abdominal pain, nausea, anorexia, diarrhea and GI bleeding.
Biopsy of enlarged lymph nodes, endoscopy or CT-Scan establishes the
diagnosis of PTLD. Primary EBV infection with high viral load after
transplantation is a known risk factor for PTLD. PTLD risk factors
include recipient pretransplant EBV negative serostatus, type of
transplant, intensity of immunosuppression, and age. Management of PTLD
involves medical reduction in immunosuppression, radiation,
chemotherapy, alfa-interferon, and use of monoclonal antibodies
(Rituximab). Mortality is much higher in children with abdominal PTLD
than those with extraabdominal disease.
References:
1- Dharnidharka VR, Sullivan EK, Stablein DM, Tejani AH, Harmon
WE; North American Pediatric Renal Transplant Cooperative Study
(NAPRTCS): Risk factors for posttransplant lymphoproliferative disorder
(PTLD) in pediatric kidney transplantation: a report of the North
American Pediatric Renal Transplant Cooperative Study (NAPRTCS).
Transplantation. 71(8):1065-8, 2001
2- Serinet MO, Jacquemin E, Habes D, Debray D, Fabre M, Bernard
O: Anti-CD20 monoclonal antibody (Rituximab) treatment for Epstein-Barr
virus-associated, B-cell lymphoproliferative disease in pediatric liver
transplant recipients. J Pediatr Gastroenterol Nutr. 34(4):389-93, 2002
3- Pearlman LS: Posttransplant viral syndromes in pediatric patients: a review. Prog Transplant. 12(2):116-24, 2002
4- Lundell R, Elenitoba-Johnson KS, Lim MS: T-cell
posttransplant lymphoproliferative disorder occurring in a pediatric
solid-organ transplant patient. Am J Surg Pathol. 28(7):967-73, 2004
5- Everly MJ, Bloom RD, Tsai DE, Trofe J: Posttransplant lymphoproliferative disorder. Ann Pharmacother. 41(11):1850-8, 2007
6- Tai CC, Curtis JL, Szmuszkovicz JR, Horn MV, Ford HR, Woo MS,
Wang KS: Abdominal involvement in pediatric heart and lung transplant
recipients with posttransplant lymphoproliferative disease increases
the risk of mortality. J Pediatr Surg. 43(12):2174-7, 2008
PSU Volume 32 No 04 APRIL 2009
Vagal Nerve Stimulator
Repetitive electrical stimulation of
the vagus nerve in the neck by using a programmable stimulator similar
to a cardiac pacemaker has been used as treatment for intractable
epilepsy in children and adults. Introduced in USA in 1988, the
treatment is based on animal experiments demonstrating that
intermittent stimulation of the vagal nerve could prevent or reduce the
frequency and/or duration of seizures. Most of these patients had
partial seizures for which resective epilepsy surgery was not feasible
or had failed, but efficacy of vagal stimulation appears to be the same
for both partial and generalized epilepsy. Vagal nerve stimulation
(VNS) is FDA approved. The device is implanted subcutaneously in the
left neck/chest and sends intermittent impulses to the left vagus nerve
through communicating leads. VNS provide relief to the patient with a
seizure disorder by decreasing the overall number and severity of
seizure activities. Complications include those of the procedure such
as wound hematoma, seroma or pocket infection, hardware failure and
those associated with stimulation of the vagal nerve such as
laryngopharyngeal dysfunction causing voice disturbance during device
activation, hoarseness, dysphagia and torticollis. Some children
might get sleep-disordered breathing (apnea) after stimulator
implantation. Mean reduction in seizures is 50%, with children
with partial complex and catastrophic epilepsy as best
responders.
References:
1- McLachlan RS: Vagus nerve stimulation for intractable epilepsy: a review. J Clin Neurophysiol. 14(5):358-68, 1997
2- FineSmith RB, Zampella E, Devinsky O: Vagal nerve stimulator: a new
approach to medically refractory epilepsy. N J Med. 96(6):37-40, 1999
3- Zalvan C, Sulica L, Wolf S, Cohen J, Gonzalez-Yanes O, Blitzer A:
Laryngopharyngeal dysfunction from the implant vagal nerve stimulator.
Laryngoscope. 113(2):221-5, 2003
4- Smyth MD, Tubbs RS, Bebin EM, Grabb PA, Blount JP: Complications of
chronic vagus nerve stimulation for epilepsy in children. J Neurosurg.
99(3):500-3, 2003
5- Hsieh T, Chen M, McAfee A, Kifle Y: Sleep-related breathing disorder
in children with vagal nerve stimulators. Pediatr Neurol. 38(2):99-103,
2008
6- Zamponi N, Rychlicki F, Corpaci L, Cesaroni E, Trignani R: Vagus
nerve stimulation (VNS) is effective in treating catastrophic 1
epilepsy in very young children. Neurosurg Rev. 31(3):291-7, 2008
Laser Depilation
Removal of hair using laser known as
laser epilation (or depilation) is an FDA approved safe technique with
consistent and long-lasting effects. It is a nearly painless procedure
that can be performed in the outpatient setting with minimal morbidity.
Most adults and children patients that utilized laser hair removal are
for cosmetic reason, especially unwanted hair. The mode of action of
laser depilation is that of selective photothermolysis of the
melanin-rich structures. Melanin within the hair is used as a natural
chromophore. It is postulated that photothermal damage destroys the
hair itself and also key cells surrounding the hair follicle to prevent
regrowth. Some clinical indications for hair removal in children
consist of hirsutism, polycystic ovarian syndrome, congenital
melanocytic nevus, generalized hypertrichosis, nevoid hypertrichosis
and pilonidal sinus disease. In pilonidal disease the intergluteal
cleft hair is removed creating an effective adjunctive therapy that
reduces recurrence. Laser hair removal is associated with a low
incidence of side effects, is painless, targets hair selectively, is
fast and can treat an area of 50 cm2 in less than a minute. The highest
incidence of side effects is seen in patients with darker skin treated
with the long-pulsed ruby laser. When administered appropriately, laser
hair removal is safe and well tolerated in children aged <16 years.
References:
1- Gault DT, Grobbelaar AO, Grover R, Liew SH, Philp B, Clement RM,
Kiernan MN: The removal of unwanted hair using a ruby laser. Br J Plast
Surg. 52(3):173-7, 1999
2- Morley S, Gault D: Hair removal using the long-pulsed ruby laser in children. J Clin Laser Med Surg. 18(6):277-80, 2000
3- Lanigan SW: Incidence of side effects after laser hair removal. J Am Acad Dermatol. 49(5):882-6, 2003
4- Rajpar SF, Hague JS, Abdullah A, Lanigan SW: Hair removal with the
long-pulse alexandrite and long-pulse Nd:YAG lasers is safe and well
tolerated in children. Clin Exp Dermatol. Jan 23, 2009
5- Lukish JR, Kindelan T, Marmon LM, Pennington M, Norwood C: Laser
epilation is a safe and effective therapy for teenagers with pilonidal
disease. J Pediatr Surg. 44(1): 282-285, 2009
Palliative Care
Palliative care is a new medical
subspecialty focused on relief of pain, symptoms and stress of serious
illness. The goal is to ensure the highest quality of life possible for
patients and their families. Palliative medicine manages serious
illness regardless of prognosis, and patients can receive it at any
point in their illness, with or without curative treatment. Palliative
care physicians objectives are to: 1) provide relief from pain or other
distressing symptoms, 2) affirm life and regard dying as a normal
process, 3) intend neither to hasten or postpone death, 4) integrate
the psychological and spiritual aspects of patient care, 5) offer
support to help patients live as actively as possible until death, 6)
offer support to help family cope during the patient's illness, 7) use
a team approach to address this needs including counseling, 8) enhance
quality of life which may influence positively the course of illness
and investigate to better understand and manage distressing clinical
complications. Palliative surgery are procedures aimed at alleviation
of patient symptoms and improvement of patient quality of life with
minimum anticipated impact on overall patient survival. Clinical
bioethical and end of life issues are being incorporated in the
instructive curriculum for competency based-training of medical schools
and postgraduate education.
References:
1- Adolph M, Dunn GP: Postgraduate Palliative Medicine training for the
surgeon: An Update on ABMS subspecialty certification. Bulletin ACS.
94(2): 6-13, 2009
2- Schiffman JD, Chamberlain LJ, Palmer L, Contro N, Sourkes B, Sectish
TC: Introduction of a pediatric palliative care curriculum for
pediatric residents. J Palliat Med. 11(2):164-70, 2008
3- McCabe ME, Hunt EA, Serwint JR: Pediatric residents' clinical and
educational experiences with end-of-life care.
Pediatrics.121(4):e731-7, 2008
3- Chiu PP, Hilliard RI, Azzie G, Fecteau A: Experience of moral
distress among pediatric surgery trainees. J Pediatr Surg.
43(6):986-93, 2008
4- Baughcum AE, Gerhardt CA, Young-Saleme T, Stefanik R, Klopfenstein
KJ: Evaluation of a pediatric palliative care educational workshop for
oncology fellows. Pediatr Blood Cancer. 49(2):154-9, 2007
PSU Volume 32 No 05 MAY 2009
Pancreatic Duct Transection
Trauma to the pancreas occurs most
commonly in children after blunt injury to the abdomen. The pancreas is
a retroperitoneal organ closely related to the lumbar vertebral column
susceptible to crush injury or transection after a direct blow to the
abdomen. Causes of pancreatic injury include motor vehicle accidents,
bicycle handbars, falls, crush or child abuse. The child develops
abdominal pain, tenderness, leukocytosis and hyperamylasemia. Diagnosis
is confirmed with CT Scan enabling the physician to grade the injury as
1 (minor contusion), 2 (major contusion), 3, (distal transection or
ductal injury), 4 (proximal transection involving the papilla), and 5
(complete pancreatic head disruption). MRCP can further delineate the
pancreatic duct injury. Most pancreatic injuries, including duct
transection, can be nonoperative managed unless the child presents with
hemodynamic instability from blood loss or associated bowel
perforation. Using nonoperative management the child is placed NPO, NG
suction, hydrated, TPN, and antibiotic prophylaxis while the pancreas
is monitored for pseudocyst development which occurs in almost 50% of
children after duct transection. Approximately half of these
pseudocysts need drainage and of these drainage procedures half are
performed percutaneously. Internal drainage procedures need wall
maturity which occurs six weeks cyst development. Follow-up CT can
display atrophy of body and tail of pancreas from enzymatic
autodigestion.
References:
1- Shilyanski J, Sena LM, Kreller M, Chait P, Babyn PS, Filler RM,
Pearl RH: Nonoperative Management of Pancreatic Injuries in Children. J
Pediatr Surg 33(2): 343-349, 1998
2- Kouchi K, Tanabe M, Yoshida H, et al: Nonoperative Management of
Blunt Pancreatic Injury in Childhood. J Pediatr Surg 34(11): 1736-1739,
1999
3- Wales PW, Shuckett B, Kim PCW: Long-Term Outcome After Nonoperative
Management of Complete Traumatic Pancreatic Transection in Children. J
Pediatr Surg 36(5): 823-827, 2001
4- Blaauw I, Winkelhorst JT, Rieu PN, et al: Pancreatic Injury in
Children: good outcome of nonoperative treatment. J Pediatr Surg 43(9):
1640-1643, 2008
Paucity of Bile Ducts
Paucity of interlobular bile ducts
(PIBD) is defined as the reduction in the number of interlobular bile
ducts. It is a cause of cholestatic jaundice during infancy sometimes
difficult to distinguish from biliary atresia. Usually, two types of
PIBD, syndromic and nonsyndromic are considered. In the syndromic type
known as Alagille's syndrome, paucity is a major feature of the
disease. In the nonsyndromic, paucity is only a part of the disease,
and an inconstant finding. Alagille's syndrome is characterized by
cholestasis of variable severity with PIBD and anomalies of the
cardiovascular system, skeleton, eyes, and face. In both types of
PIBD the diagnosis is made with the use of liver biopsy while
certifying patency of the extrahepatic biliary system through HIDA,
MRCP or intraoperative cholangiography. Therapy consists of
supplementation of those vitamins and administration of cholestyramine,
phenobarbital, prednisolone, or ursodeoxycholic acid. Children with
Alagille identified in infancy because of cholestasis have a 50%
probability of long-term survival without liver transplantation.
Factors that contribute to mortality are complex heart disease,
intracranial bleeding, hepatic disease or hepatic transplantation.
Prognosis of the nonsyndromic type is variable.
References:
1-Hadchouel M: Paucity of interlobular bile ducts. Semin Diagn Pathol. 9(1):24-30, 1992
2- Elmslie FV, Vivian AJ, Gardiner H, Hall C, Mowat AP, Winter RM:
Alagille syndrome: family studies. J Med Genet. 32(4):264-8, 1995
3- Hoffenberg EJ, Narkewicz MR, Sondheimer JM, Smith DJ, Silverman A,
Sokol RJ: Outcome of syndromic paucity of interlobular bile ducts
(Alagille syndrome) with onset of cholestasis in infancy. J Pediatr.
127(2):220-4, 1995
4- Koçak N, Gürakan F, Yüce A, Caglar M, Kale G,
Gögüs S: Nonsyndromic paucity of interlobular bile ducts:
clinical and laboratory findings of 10 cases. J Pediatr Gastroenterol
Nutr. 24(1):44-8, 1997
5- Emerick KM, Rand EB, Goldmuntz E, Krantz ID, Spinner NB, Piccoli DA:
Features of Alagille syndrome in 92 patients: frequency and relation to
prognosis. Hepatology. 29(3):822-9, 1999
6- Wang JS, Wang XH, Zhu QR, Wang ZL, Hu XQ, Zheng S: Clinical and
pathological characteristics of Alagille syndrome in Chinese children.
World J Pediatr. 4(4):283-8, 2008
Double Cystic Duct
The biliary tree is composed of
intrahepatic radicals, common hepatic duct, gallbladder, a single
cystic duct and a common bile duct emptying into the papilla of Vater.
Anatomic variations of the biliary tree are common and a cause of
biliary injury during removal of both gallbladder and common bile duct
stones. Variants of the extrahepatic bile ducts are present in 10% of
patients such as low insertion of the cystic duct into the common
hepatic duct, emptying of the cystic duct into the right hepatic duct
and a second-order large branch draining into the cystic duct. One of
the most rare congenital anomaly of the biliary tree is the presence of
double cystic duct. It is usually associated with a double gallbladder
(80%). Less than 15 cases have been reported in the literature, all
adults, none in children. Recently we encountered the first case of
double cystic duct associated with a single gallbladder in a
six-year-old child. Most cases have been identified during open
cholecystectomy for symptomatic cholelithiasis. The cystic ducts
usually drain to the common bile duct and the right hepatic duct.
Other time the two cystic ducts form a triangular formation with the
common hepatic duct. There is only one cystic artery that arises from
the right hepatic artery and accompanies the primary cystic duct to be
distributed to the gallbladder. This rare anatomic variant can be
defined using intraoperative cholangiography whenever the doubt occurs
or during preoperative ERCP or MRCP studies. Management is ligation of
both ducts.
References:
1- Hirono Y, Takita Y, Nitta N, Hashimoto H: Double cystic duct found
by intraoperative cholangiography in laparoscopic cholecystectomy. Surg
Laparosc Endosc. 7(3):263-5, 1997
2- Lamah M, Dickson GH: Congenital anatomical abnormalities of the
extrahepatic biliary duct: a personal audit. Surg Radiol Anat.
21(5):325-7, 1999
3- Tsutsumi S, Hosouchi Y, Shimura T, Asao T, Kojima T, Takenoshita S,
Kuwano H: Double cystic duct detected by endoscopic retrograde
cholangiopancreatography and confirmed by intraoperative
cholangiography in laparoscopic cholecystectomy: a case report.
Hepatogastroenterology. 47(35):1266-8, 2000
4- Paraskevas G, Papaziogas B, Natsis K, Spanidou S, Kitsoulis P,
Atmatzidis K, Tsikaras P: An accessory double cystic duct with single
gallbladder. Chirurgia (Bucur). 102(2):223-5, 2007
5- De Filippo M, Calabrese M, Quinto S, Rastelli A, Bertellini A,
Martora R, Sverzellati N, Corradi D, Vitale M, Crialesi G, Sarli L,
Roncoroni L, Garlaschi G, Zompatori M: Congenital anomalies and
variations of the bile and pancreatic ducts: magnetic resonance
cholangiopancreatography findings, epidemiology and clinical
significance. Radiol Med. 113(6):841-59, 2008
6- Huston TL, Dakin GF: Double cystic duct. Can J Surg, Vol. 51(1): E9-E10, 2008
PSU Volume 32 No 06 JUNE 2009
Haddad Syndrome
Haddad syndrome is a rare disorder
considered a neurocristopathy, a set of disease processes characterized
by maldevelopment of the neural crests. Neurocristopathies are a group
of diverse disorders resulting from defective growth, differentiation,
and migration of the neural crest cells. Children with Haddad syndrome
present with the combination of congenital central hypoventilation
syndrome (also known as Ondine's curse), and Hirschsprung's disease
(HD). Almost 10% of these cases of Hirschsprung's disease have total
intestinal aganglionosis. The initial clinical manifestation in the
neonatal period is apnea of no identifiable cause followed by
constipation or bowel obstruction. Strong clinical suspicion, rectal
biopsy and genetic mutation detection makes the diagnosis of Haddad
syndrome. Other associated features includes ophthalmic abnormalities,
esophageal dysmotility, sensorineural hearing loss, neural crest tumors
and signs and symptoms of autonomic nervous system dysfunction. A
genetic basis for Haddad syndrome has been suggested associated with a
mutations detection rate above 90% in chromosome 4p12 PHOX2B gene.
Inheritance is autosomal dominant. Management consist of tracheotomy,
home ventilatory support, TPN, proximal decompressive ostomy and long
small bowel myectomy-myotomy. The prognosis is poor specially in
underdeveloped countries.
References:
1- Shahar E, Shinawi M: Neurocristopathies presenting with neurologic
abnormalities associated with Hirschsprung's disease. Pediatr Neurol.
28(5):385-91, 2003
2- D'Souza S, Khubchandani RP: Haddad syndrome--congenital central
hypoventilation associated with Hirschsprung's disease. Indian J
Pediatr. 70(7):597-9, 2003
3- Bajaj R, Smith J, Trochet D, Pitkin J, Ouvrier R, Graf N, Sillence
D, Kluckow M: Congenital central hypoventilation syndrome and
Hirschsprung's disease in an extremely preterm infant. Pediatrics.
115(6):e737-8, 2005
4- Dejhalla M, Parton P, Golombek SG: Case report of Haddad syndrome in
a newborn: congenital central hypoventilation syndrome and
Hirschsprung's disease. J Perinatol. 26(4):259-60, 2006
5- Lai D, Schroer B: Haddad syndrome: a case of an infant with central congenital hypoventilation
syndrome and Hirschsprung disease. J Child Neurol. 23(3):341-3, 2008
6- Otabor IA, Balint JP, Besner GE: Myectomy-myotomy for long segment
Hirschsprung's disease in a patient with Haddad syndrome. J Pediatr
Surg. 44(3):620-2, 2009
Disc Cell Battery Ingestion
Preschool children and toddlers enjoy
taking things from their hand to their mouth. This includes disc,
button or coin cell batteries with more than 3000 coin cell battery
ingestion reported yearly in the United States. Button batteries are
being used with increasing frequency in a variety of devices including
hearing aids, watches and calculators. Most of these ingested foreign
bodies will pass the gastrointestinal tract without causing harm, but a
few will produce a very serious complication. Such rare complications
include esophageal perforation & stricture, aortoesophageal
fistula, gastric perforation, tracheoesophageal fistula and vocal cord
paralysis. The tissue damage that result from contact with charged
battery is a chemical burn caused by production of sodium hydroxide
(cathode) and hydrochloric acid (anode) generated from electric current
passing through physiologic electrolyte solution. The alkaline burn
with liquefaction necrosis, fat saponification and inflammatory cell
infiltration causes the most severe histologic injury. It's not caused
by the content of the battery or pressure necrosis changes. Coin cell
batteries differ from coin currency in simple x-rays. If the battery
impacts in the esophagus or hypopharynx, emergency endoscopic
management is necessary. Once in the stomach, the battery will usually
pass through the gastrointestinal tract without long-term
complications. Its passage can be monitored with serial radiographs.
References:
1- Kost KM, Shapiro RS: Button battery ingestion: a case report and
review of the literature. J Otolaryngol. 16(4):252-7, 1987
2- Maves MD, Carithers JS, Birck HG: Esophageal burns secondary to disc
battery ingestion. Ann Otol Rhinol Laryngol. 93(4 Pt 1):364-9, 1984
3- Slamon NB, Hertzog JH, Penfil SH, Raphaely RC, Pizarro C, Derby CD:
An unusual case of button battery-induced traumatic tracheoesophageal
fistula. Pediatr Emerg Care. 24(5):313-6, 2008
4- Hamilton JM, Schraff SA, Notrica DM: Severe injuries from coin cell
battery ingestions: 2 case reports. J Pediatr Surg. 44(3):644-7, 2009
5- Litovitz T, Schmitz BF: Ingestion of cylindrical and button
batteries: an analysis of 2382 cases. Pediatrics. 89(4 Pt 2):747-57,
1992
Proteus Syndrome
Proteus syndrome (PS) is a rare
congenital hamartomatous syndrome that causes sporadic overgrowth of
multiple tissues in a patchy or mosaic pattern. The overgrowth can
involve skin, subcutaneous tissue, connective tissue (including bone),
the central nervous system, and viscera. Complications of PS include
progressive skeletal deformities, plantar gigantism of the hands and
feet, invasive lipomas, benign and malignant tumors, and deep venous
thrombosis with pulmonary embolism. The name Proteus comes from a Greek
mythical sea god who was able to change his body form freely. The
disease process is not usually apparent at birth but develops rapidly
in childhood. Common manifestations include macrodactyly, vertebral
abnormalities, asymmetric limb overgrowth and length discrepancy,
hyperostosis, abnormal and asymmetric fat distribution, asymmetric
muscle development, connective-tissue nevi, and vascular malformations.
Diagnosis and management of the disease depend heavily on clinical
evaluation and imaging using strict criteria. Histopathological
features of lesions resected from children with PS predominantly
include hamartomatous mixed connective tissue lesions, benign neoplasms
such as lipomas, and lymphatic-rich vascular malformations. Potential
complications such as difficult intubation, pulmonary hypertension, and
pulmonary thromboembolism necessitates careful preoperative and
anesthetic preparation.
References:
1- Biesecker LG: The multifaceted challenges of Proteus syndrome. JAMA. 285(17):2240-3, 2001
2- Jamis-Dow CA, Turner J, Biesecker LG, Choyke PL: Radiologic
manifestations of Proteus syndrome. Radiographics. 24(4):1051-68, 2004
3- Cekmen N, Kordan AZ, Tuncer B, Gungor I, Akcabay M: Anesthesia for proteus syndrome. Paediatr Anaesth. 14(8):689-92, 2004
4- Biesecker L: The challenges of Proteus syndrome: diagnosis and management. Eur J Hum Genet. 14(11):1151-7, 2006
5- Hoey SE, Eastwood D, Monsell F, Kangesu L, Harper JI, Sebire NJ:
Histopathological features of Proteus syndrome. Clin Exp Dermatol.
33(3):234-8, 2008
6- Furquim I, Honjo R, Bae R, Andrade W, Santos M, Tannuri U, Kim C:
Proteus syndrome: report of a case with recurrent abdominal
lipomatosis. J Pediatr Surg. 44(4):E1-3, 2009