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.

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.

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.

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.

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.

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.

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.

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.

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.

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. 

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.

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.  

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.

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.

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.

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.

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.

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.

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

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