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.

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.  

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.

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.

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.

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.

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.

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.

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


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. 

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.

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.

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. 

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 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.

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. 

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.   

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.

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.    

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. 

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

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