VOLUME 46, 2016

PSU Volume 46 NO 01 JANUARY 2016

Congenital Paraesophageal Hernia

Congenital paraesophageal hernia (CPH) is the rarest of all diaphragmatic hernia of childhood. As the name implies, the patient is born with the defect. Most paraesophageal hernias are acquired after procedures performed in the GE junction (antireflux procedures). In CPH the defect can go unnoticed for several years or present with recurrent chest infections or vague gastrointestinal symptoms. Left alone CPH can cause high morbidity or even mortality hence the importance of an early diagnosis. CPH occurs because of absent or abnormally lax anatomic anchors of the stomach and gastroesophageal junction associated to a paraesophageal defect. The fundus is pushed through the defect by increase intraabdominal pressure entering the thoracic cavity causing lung compression and mediastinal shift. If this progress the antrum also enters the thorax and organoaxial volvulus of the stomach occur causing gastric obstruction and esophageal dilatation. With enlargement of the defect bowel and omentum can herniate. There is absence of pulmonary hypoplasia. Clinically PEH can present acutely with incarceration, obstruction, gangrene, perforation, bleeding, anemia, as well as acute respiratory complications. Other times the child has chronic chest infections with intermittent vomiting. Initial work-up should include chest film, upper gastrointestinal series and abdominal CT-Scan to determine herniated content and detect complications of affected lungs. Associated anomalies with CPH include short esophagus and microgastria. Surgical repair should be done promptly to avoid serious complications. The procedure can be performed open or laparoscopically and aims to resect the hernia sac, reduce the herniated content, close the hiatal defect, anchor the stomach or do a partial antireflux procedure, and check for associated malformations.

1- Al-Salem AH: Congenital paraesophageal hernia in infancy and childhood. Saudi Med J. 21(2):164-7, 2000
2- Imamoglu M, Cay A, Kosucu P, Ozdemir O, Orhan F, Sapan L, Sarihan H: Congenital paraesophageal hiatal hernia: pitfalls in the diagnosis and treatment.  J Pediatr Surg. 40(7):1128-33, 2005
3- Jetley NK, Al-Assiri AH, Al Awadi D: Congenital para esophageal hernia: a 10 year experience from Saudi Arabia. Indian J Pediatr. 76(5):489-93, 2009
4- Priego Jimenez P, Salvador Sanches JL, Angel V, Escrig-Sos J: Short-term results for laparoscopic repair of large paraesophageal hiatal hernias with Gore Bio mesh. Int J Surg. 12(8):794-7, 2014
5- Athanasakis H, Tzortzinis A, Tsiaoussis J, Vassilakis JS, Xynos E: Laparoscopic repair of paraesophageal hernia. Endoscopy. 33(7):590-4, 2001
6- Yousef Y, Lemoine C, St-Vil D, Emil S: Congenital paraesophageal hernia: The Montreal experience. J Pediatr Surg. 50(9):1462-6, 2015

Intradiploic Dermoid Cysts

Dermoid cysts are congenital benign neoplasms derived from both ectoderm and mesoderm tissue layers. They typically occur in childhood during the first or second decade of life. They are composed of a keratinizing squamous epithelium present together with dermal derivatives such as hair follicles, smooth muscle, sweat gland, sebaceous gland and fibroadipose tissue. On-tenth of all dermoid cysts occurs in the region of the head and neck in locations such as periorbital, nasal, frontotemporal, submental and suprasternal. Dermoid cysts of the head and neck are thought to be the congenital inclusion type. The periorbital region is the most commonly reported site for dermoid cysts of head and neck, most commonly near the zygomaticofrontal suture, laterals to the lateral canthus. Some dermoid cysts exhibit intermittent discharge of sebaceous material or recurrent local infection. In the temporal bone dermoid cysts can be mistakenly labels as a first branchial cleft or pouch anomalous cyst. Lesions in the midline of the cranium are more concerning for intracranial extension. Dermoid cysts that are located in the nasal bridge or temporal area, produce recurrent infection or exhibit a draining sinus should alert the physician toward the presence of intracranial extension warranting further imaging with CT-Scan or MRI to plan excision. Intradiploic dermoid cysts are a rare subtype of the frontotemporal dermoids occurring most commonly at the bregma. They may appear as a lytic defect in the skull. The overwhelming majority of periorbital and frontotemporal dermoids are superficially located and amenable to treatment with simple excision.  Complete en bloc surgical resection with careful follow-up is the treatment of choice for dermoid cysts. 

1- Parag P, Prakash PJ, Zachariah N: Temporal dermoid--an unusual presentation. Pediatr Surg Int. 17(1):77-9, 2001
2- Scolozzi P, Lombardi T, Jaques B: Congenital intracranial frontotemporal dermoid cyst presenting as a cutaneous fistula. Head Neck. 27(5):429-32, 2005
3- Pryor SG, Lewis JE, Weaver AL, Orvidas LJ: Pediatric dermoid cysts of the head and neck. Otolaryngol Head Neck Surg. 132(6):938-42,2005
4- Rinna C, Reale G, Calafati V, Calvani F, Ungari C: Dermoid cyst: unusual localization. J Craniofac Surg. 23(5):e392-4, 2012
5- Vega RA, Hidlay DT, Tye GW, Fuller CE, Rhodes JL: Intradiploic dermoid cyst of the lateral frontotemporal skull: case report and review of the literature. Pediatr Neurosurg. 49(4):232-5, 2013
6- Barnard AR, Jones AP, Hodgkinson PD, Jenkins AJ: Beware frontotemporal dermoids - they may have intracranial extension: a case of  a middle cranial fossa cyst.  J Plast Reconstr Aesthet Surg. 65(7):e185-8, 2012

Esophageal Atresia with Right Aortic Arch

Almost 5% of children with esophageal atresia (EA) with or without an associated tracheoesophageal fistula have a right aortic arch (RAA). Preoperative localization of the aortic arch position previous to repair of EA is of utmost importance to avoid an extremely difficult right thoracotomy. Preoperative localization of a RAA using echocardiography correctly identifies the arch position in two-thirds of patients. The surgeon needs to reliable identify the position of the aortic arch, the laterality of the descending thoracic aorta and the nature of any associated congenital heart disease. If an aortic arch pass over the right mainstem bronchus, it will descend through the right side of the chest 95% of the time, and there will be complete absence of any retro-esophageal component to the aorta. Most will have mirror-image branching of the innominate and subclavian arteries. Infants with EA with RAA have more vascular rings, cardiac anomalies and operative complications compared with those with LAA. Up to 50% of children with EA and RAA have associated cardiac malformations, especially ventricular and atrial septal defects. RAA is found in 25% of cases of Tetralogy of Fallot. Should the suspicion of a RAA in EA arise, further diagnostic evaluation using CT-angio or MRI will elucidate the exact anatomic variant. When RAA is associated with EA, the gap length between the esophageal pouches is usually increased. A left thoracotomy in the face of RAA allows one to gain better exposure to the esophagus and tracheoesophageal fistula without the obstruction of the aorta. In addition, owing to the type of vascular ring that can be encountered the left-sided thoracotomy allows access to ligate the PDA or manage the vascular ring. Should a RAA be unexpectedly encountered during a right thoracotomy and the anastomosis is not technically feasible a delayed left thoracotomy permits more accurate evaluation of the vascular anatomy.  

1- Bowkett B, Beasley SW, Myers NA: The frequency, significance, and management of a right aortic arch in association with esophageal atresia. Pediatr Surg Int. 15(1):28-31, 1999
2- Babu R, Pierro A, Spitz L, Drake DP, Kiely EM: The management of oesophageal atresia in neonates with right-sided aortic arch.  J Pediatr Surg. 35(1):56-8, 2000
3- Kosloske AM, Jewell PF: A right-sided aortic arch in 12 infants with esophageal atresia. J Pediatr Surg. 35(11):1684, 2000
4- Allen SR, Ignacio R, Falcone RA, Alonso MH, Brown RL, Garcia VF, Inge TH, Ryckman FC, Warner BW, Azizkhan RG, Tiao GM: The effect of a right-sided aortic arch on outcome in children with esophageal atresia and tracheoesophageal fistula. J Pediatr Surg. 41(3):479-83, 2006
5- Bicakci U, Tander B, Ariturk E, Rizalar R, Ayyildiz SH, Bernay F: The right-sided aortic arch in children with esophageal atresia and tracheo-esophageal fistula: a repair through the right thoracotomy. Pediatr Surg Int. 25(5):423-5, 2009
6- Wood JA, Carachi R: The right-sided aortic arch in children with oesophageal atresia and tracheo-oesophageal fistula. Eur J Pediatr Surg. 22(1):3-7, 2012
7- Paraloni F, Armellini A, Boroni G, Bagolan P, Alberti D: The management of newborns with esophagea atresia and right aortic arch: A systematic review or still unsolved problem. J Pediatr Surg  2015 (Article in press).

PSU Volume 46 NO 02 FEBRUARY 2016

Body Stalk Anomaly

Anterior abdominal wall defects are classified as gastroschisis, omphalocele and body-stalk anomalies. Body stalk anomaly (BSA) is the most rare and a severe abdominal wall defect associated with a high incidence of spontaneous abortion. BSA consists of a large abdominal wall defect in more severe cases of thoracic organs as well, associated with severe kyphoscoliosis and a very short or even absent umbilical cord. In addition the upper half of the fetal body is in the amniotic cavity while the lower half is in the celomic cavity. This anomaly might also occur in conjunction with neural tube defects, genitourinary malformations, abnormalities of the chest wall, intestinal atresia, and craniofacial defects. The most plausible theory of formation of BSA is that early amnion rupture before obliteration of the celomic cavity is the underlying mechanism. The rupture results in the expulsion of the embryo or a part of it into the exocoelomic cavity. Other alternative theories include an abnormal embryonic folding resulting in the failure of the celomic cavity to obliterate or vascular disruption during the first 4-6 weeks of gestation. Most cases of body stalk syndrome are incompatible with life. Body stalk anomaly is not associated with chromosomal abnormalities and there is no increased risk for a recurrence. Levels of alpha-fetoprotein in maternal serum are very high in BSA. Prenatal ultrasound in the 9th to 10th weeks of pregnancy has detected this diagnosis. It has been reported that 50% of women with fetuses affected by body stalk anomaly smoke cigarettes or drink alcohol and 30% of them smoked marijuana. Continuation of pregnancy in the presence of BSA is associated with greater maternal risks, in the form of labor complications requiring cesarean section. Prenatal diagnosis enables prompt knowledge of the fetus condition permitting appropriate counseling and management.  

1- Daskalakis G, Pilalis A, Papadopoulos D, Antsaklis A: Body stalk anomaly diagnosed in the 2nd trimester. Fetal Diagn Ther. 18(5):342-4, 2003
2- Hirokawa S, Uotani H, Futatani T, Sasaki Y, Ogawa J, Sakai M, Tsukada K, Saito S: A case of body stalk anomaly arising in the second baby of a triplet pregnancy after in-vitro fertilization and embryo transfer. Pediatr Surg Int. 19(3):223-5, 2003
3- Kanamori Y, Hashizume K, Sugiyama M, Tomonaga T, Takayasu H, Ishimaru T, Terawaki K, Suzuki K, Goishi K, Takamizawa M: Long-term survival of a baby with body stalk anomaly: report of a case. Surg Today. 37(1):30-3, 2007
4- Murphy A, Platt LD: First-trimester diagnosis of body stalk anomaly using 2- and 3-dimensional
sonography. J Ultrasound Med. 30(12):1739-43, 2011
5- Costa ML, Couto E, Furlan E, Zaccaria R, Andrade K, Barini R, Nomura ML: Body stalk anomaly: adverse maternal outcomes in a series of 21 cases. Prenat Diagn. 32(3):264-7, 2012
6- Kocherla K, Kumari V, Kocherla PR:Prenatal diagnosis of body stalk complex: A rare entity and review of literature. Prenatal diagnosis of body stalk complex: A rare entity and review of literature. Indian J Radiol Imaging. 25(1): 67–70, 2015

Transverse Colonic Volvulus

Around 5% of all intestinal obstructions are caused by colonic volvulus. Sigmoid colon is the most common site for volvulus followed by the cecum, transverse and splenic flexure in order of frequency. Volvulus of the transverse colon is a very rare event in children. Mean age is ten years. Main reason is that the broad-based short mesentery normally prevents the transverse colon from rotating either clockwise or anti-clockwise. Transverse colonic volvulus (TCV) is more common in Eastern Europe, Scandinavian countries and Japan. Risk of volvulus is increased by the following factors: elongation and redundancy of the transverse colon, narrowing of the mesenteric attachments causing flexures to come together, and absence or malfixation of the mesenteries. Other mechanical causes include previous volvulus of transverse or sigmoid, distal colonic obstruction, adhesions, malposition of the colon after surgery, inflammatory structures and carcinoma. Chronic constipation, cerebral palsy, mental retardation, Hirschsprung's disease and high fiber diet is a characteristic found in children with TCV.  Symptoms of TCV include abdominal pain, vomiting, abdominal distension and tenderness. Clinically TCV presents acutely and fulminant or subacute progressive depending on the degree of ischemia. Simple films show distension of proximal colon, empty distal bowel and two air-fluids levels caused by double closed loop obstruction. A barium enema might show a "bird beak" or "ace of spades" at the focus of the twist bowel. CT Scan is diagnostic. Management consists of manual detorsion, detorsion with colopexy, resection with primary anastomosis in case of significant ischemia, or resection with colostomy and/or ileostomy and mucous fistula in cases of intraoperative hemodynamic instability. Manual detorsion is associated with a high incidence of recurrence. Resection with or without primary anastomosis is the treatment of choice to prevent recurrence.

1- Asabe K, Ushijima H, Bepu R, Shirakusa T: A case of transverse colon volvulus in a child and a review of the literature in  Japan. J Pediatr Surg. 37(11):1626-8, 2002
2- Sparks DA, Dawood MY, Chase DM, Thomas DJ: Ischemic volvulus of the transverse colon: A case report and review of literature. Cases J. 1(1):174, 2008
3- Rahbour G, Ayantunde A, Ullah MR, Arshad S, Kerwat R: Transverse colon volvulus in a 15 year old boy and the review of the literature. World J Emerg Surg. 5:19, 2010
4- Sage MJ, Younis J, Schwab KE, Galbraith KA: Colopexy as a treatment option for the management of acute transverse colon volvulus: a case report.  J Med Case Rep. 6:151, 2012
5- Smith C, Cho A, Tsang T: Transverse colonic volvulus in a child: successful management with decompression  and delayed laparoscopic colopexy. European J Pediatr Surg Rep. 1(1):48-50, 2013
6- Waluza JJ, Aronson DC, Nyirenda D, Zoetmulder FA, Borgstein ES: Transverse colon volvulus in children: A case series and a review of the literature. J Pediatr Surg. 50(10):1641-3, 2015

Povidone-Iodine Pleurodesis

Povidone-iodine is an antiseptic material used routinely to clean surgical sites and as a procedural disinfectant. It has been utilized as a safe chemical pleurodesis agent in cases of spontaneous pneumothorax, air leaks, malignant pleural effusions and chylothorax in children and adults when injected into the pleural space. The mechanism of action of povidone-iodine appears to be related to chelation of proteins and enhanced sclerosis. Iodine has strong oxidative and cytotoxic properties which induce a potent inflammatory response. In addition the low pH of povidone-iodine may contribute to the local inflammatory response. When used in concentrations of 4% iodopovidone produces pronounced pleural adhesion and thickening mesothelial cell injury. The most significant complication reported is chest pain. Visual loss resulting from the breakdown of the diffusion barrier of the retinal pigment epithelium with concentrations of 10% during thoracoscopic surgery has been reported. Procedure consists of injecting a saline solution diluted 4% povidone-iodine into the pleural space at a dose of 2 ml/kg of weight followed by clamping the chest tube for four hours. Using these low iodine concentrations, neither renal function nor thyroid function is impaired both immediately after the procedure or long term. To further reduce the risk and severity of side effects, 2% povidone–iodine may be used, as it has experimentally been shown that it is as effective as 4% concentration. Multicenter randomized studies that compare the risks and benefit of povidone-iodine should be done before we label this option as the procedure of choice.

1- Mahmodlou R, Rahimi-Rad MH, Alizadeh H: Efficacy and safety of iodopovidone pleurodesis through chest tube in spontaneous pneumothorax. Pneumologia. 60(2):78-80, 2011
2- Arayici S, Simsek GK, Oncel MY, Yilmaz Y, Canpolat FE, Dilmen U: Povidone-iodine for persistent air leak in an extremely low birth weight infant.  J Pediatr Surg. 48(5):E21-3, 2013
3- Teixeira LR, Vargas FS, Puka J, Acencio MM, Antonangelo L, Terra RM, Damico FM, Pitta FG, Marchi E: Effectiveness and safety of iodopovidone in an experimental pleurodesis model. Clinics (Sao Paulo). 68(4):557-62, 2013
4- Ibrahim IM, Dokhan AL, El-Sessy AA, Eltaweel MF: Povidone-iodine pleurodesis versus talc pleurodesis in preventing recurrence of malignant pleural effusion. J Cardiothorac Surg. 10:64, 2015
5- Scottoni F, Fusaro F, Conforti A, Morini F, Bagolan P: Pleurodesis with povidone-iodine for refractory chylothorax in newborns: Personal experience and literature review.  J Pediatr Surg. 50(10):1722-5, 2015
6- Resch B, Freidl T, Reiterer F: Povidone-iodine pleurodesis for congenital chylothorax of the newborn. Arch Dis Child Fetal Neonatal Ed. 101(1):87-8, 2016

PSU Volume 46 NO 03 MARCH 2016

Phrygian Cap Gallbladder

The most common congenital anomaly of the biliary tract found in 2-6% of autopsies is a folded gallbladder, also known as Phrygian cap gallbladder, since it resembles a bonnet worn by the ancient Phrygians of Asia Minor. The Phrygian cap occurs when the fundus of the gallbladder fold on itself. The literature suggests the Phrygian cap anomaly predispose this segment of the gallbladder to slow flow or delayed filling and hence lithiasis. High definition ultrasound and nuclear studies can identify a Phrygian cap. Other studies that can establish the diagnosis include CT-Scan and MRCP. Phrygian cap gallbladder should be a differential diagnosis considered when a mass is found associated with the gallbladder or liver adjacent to the gallbladder. Phrygian cap anomaly can also simulate duplication of the gallbladder. Acalculous and calculous cholecystitis have been associated with Phrygian cap gallbladder. In many cases with "Phrygian cap" deformity, there is a mucosal fold created, which partially subdivides the lumen of the organ. Most patients with Phrygian cap gallbladder are asymptomatic. Gallbladder with Phrygian cap deformity can lead to misdiagnosis of gallbladder stones on B-mode ultrasonography. Prophylactic cholecystectomy is not indicated in patients with Phrygian cap deformity alone as it is of no pathological significance. Surgery is performed only for patients with symptoms or complications of gallbladder disease irrespective of Phrygian cap deformity.

1- Saji S, Tony J, Kumar KS, Thomas V: Phrygian cap in magnetic resonance cholangiogram. Indian J Gastroenterol. 27(5):194, 2008
2- de Csepel J, Carroccio A, Pomp A: Soft-tissue images. "Phrygian cap" gallbladder. Can J Surg. 46(1):50-1, 2003
3- Al-Ashqar M, Maliyakkal AK, Shiwani MH, Anwar S: Acalculous Phrygian cap cholecystitis. BMJ Case Rep. 2013 Oct 4;2013.
4- Rafailidis V, Varelas S, Kotsidis N, Rafailidis D: Two congenital anomalies in one: an ectopic gallbladder with phrygian cap deformity. Case Rep Radiol. 2014;2014:246476
5- Kannan NS, Kannan U, Babu CP: Congenital bilobed gallbladder with phrygian cap presenting as calculus cholecystitis. J Clin Diagn Res. 8(8):ND05-6, 2014
6- van Kamp MJ, Bouman DE, Steenvoorde P, Klaase JM: A phrygian cap. Case Rep Gastroenterol. 17;7(2):347-51, 2013

BI-RADS in Children

Breast masses in children are rare but cause significant concern in parents. Fortunately most breast masses in children are benign consisting of fibroadenomas, gynecomastia, cysts and macromastia. Size, presence of symptoms and growth are the characteristics that decide upon excision for diagnostic and therapeutic reasons. To avoid ionizing radiation, the ultrasound has become the best imaging study to perform in children breast masses. The breast imaging reporting and data system (Bi-Rads) developed by the American College of Radiology categorize these lesions based on the likehood of malignancy stipulating the need of tissue diagnosis in high classification cases. The risk of malignancy in children is lower than adults. Correlating Bi-Rads findings with tissue diagnosis it has been found that breast lesions in children with Bi-Rads classification of 4 where excision is strongly recommended are almost always benign. This usually occurs with juvenile fibroadenomas in teenage females. Most breast masses in children are commonly described as oval, hypoechoic and circumscribed, and almost never classified as calcified, with angular or spiculated margins or showing posterior shadowing. Should we follow adult management children with Bi-Rads 4 would undergo core-needle biopsy, a procedure which is painful and often requires anesthesia. In such situation excisional biopsy using a periareolar incision is more practical than core-needle biopsy. Breast masses with high Bi-Rads classification should be removed because of size, evidence of growth or persistent symptoms. Bi-Rads classification overstates the risk of malignancy in the pediatric population invalidating the treatment algorithm associated with the classification. Short term imaging follow-up is an acceptable alternative to immediate biopsy.  

1- Kwak JY, Kim EK, Park HL, Kim JY, Oh KK: Application of the breast imaging reporting and data system final assessment system in sonography of palpable breast lesions and reconsideration of the modified triple test. J Ultrasound Med. 2006 Oct;25(10):1255-61.
2- Park YM, Kim EK, Lee JH, Ryu JH, Han SS, Choi SJ, Lee SJ, Yoon HK: Palpable breast masses with probably benign morphology at sonography: can biopsy  be deferred? Acta Radiol. 2008 Dec;49(10):1104-11
3- Loving VA, DeMartini WB, Eby PR, Gutierrez RL, Peacock S, Lehman CD: Targeted ultrasound in women younger than 30 years with focal breast signs or symptoms: outcomes analyses and management implications. AJR Am J Roentgenol. 2010 Dec;195(6):1472-7.
4- Son EJ, Kim EK, Youk JH, Kim MJ, Kwak JY, Choi SH: Imaging-histologic discordance after sonographically guided percutaneous breast biopsy: a prospective observational study. Ultrasound Med Biol. 2011 Nov;37(11):1771-8
5- Xiao X, Ou B, Yang H, Wu H, Luo B: Breast contrast-enhanced ultrasound: is a scoring system feasible? A preliminary study in China. PLoS One. 2014 Aug 18;9(8):e105517
6- Koning JL, Davenport KP, Poole PS, Kruk PG, Grabowski JE: Breast Imaging-Reporting and Data System (BI-RADS) classification in 51 excised palpable pediatric breast masses.  J Pediatr Surg. 2015 Oct;50(10):1746-50.(leading article).

Asymptomatic Malrotation Revisted

Midgut volvulus is a clockwise rotation of the bowel occurring in children and adults with malrotation presenting with acute symptoms of ischemia and bowel obstruction. Early recognition and surgical intervention is mandatory. Most cases occur during the neonatal period. Not all cases of malrotation ends in midgut volvulus, hence some patients  present with asymptomatic malrotation if they are found incidentally with imaging studies compatible with such but complete absence of symptoms.  Radiologic investigation includes upper gastrointestinal series, Doppler sonography and contrast enhanced CT of the abdomen. UGIS is the test of choice for diagnosing malrotation. Ultrasound can be falsely negative and cannot be used to definitively rule out malrotation or volvulus. The management of asymptomatic or incidentally discovered malrotation is controversial. Malrotation can be divided into true malrotation with a narrow mesenteric stalk, nonrotation with a broad mesentery, and atypical defined as malposition of the ligament of Treitz or duodenum. Children with a higher incidence of malrotation include those with congenital heart disease (CHD) and heterotaxy syndrome, abdominal wall defect and diaphragmatic hernia. It's unusual to perform a Ladd's procedure for gastroschisis, omphalocele or diaphragmatic hernia as adhesions from surgery decrease the incidence of volvulus. There is minimal evidence to support screening in asymptomatic patients with CHD and heterotaxy syndrome. In this group of children it seems better to wait for palliation of the heart defect before offering a Ladd's procedure. Recent data from APSA regarding the need for prophylactic Ladd's procedure in asymptomatic malrotated children suggest it should be done for young age, while observation may be appropriate in the older asymptomatic patient. The laparoscopic approach is safe for diagnosing and managing asymptomatic malrotation, while there is minimal evidence to support the laparoscopic approach in cases of known volvulus or in neonates. Any age, any condition with symptomatic malrotation should undergo a Ladd's procedure.  

1- Dilley AV, Pereira J, Shi EC, Adams S, Kern IB, Currie B, Henry GM: The radiologist says malrotation: does the surgeon operate? Pediatr Surg Int. 16(1-2):45-9, 2000
2- Prasil P, Flageole H, Shaw KS, Nguyen LT, Youssef S, Laberge JM: Should malrotation in children be treated differently according to age? J Pediatr Surg. 5(5):756-8, 2000
3- Cohen Z, Kleiner O, Finaly R, Mordehai J, Newman N, Kurtzbart E, Mares AJ: How much of a misnomer is "asymptomatic" intestinal malrotation? Isr Med Assoc J. 5(3):172-4, 2003
4- Malek MM, Burd RS: The optimal management of malrotation diagnosed after infancy: a decision analysis. Am J Surg. 191(1):45-51, 2006
5- Lampl B, Levin TL, Berdon WE, Cowles RA: Malrotation and midgut volvulus: a historical review and current controversies in diagnosis and management. Pediatr Radiol. 39(4):359-66, 2009
6- Lodwick DL, Minneci PC, Deans KJ: Current surgical management of intestinal rotational abnormalities. Curr Opin Pediatr. 27(3):383-8, 2015
7- Graziano K, Islam S, Dasgupta R, Lopez ME, Austin M, Chen LE, Goldin A, Downard CD, Renaud E, Abdullah F: Asymptomatic malrotation: Diagnosis and surgical management: An American Pediatric Surgical Association outcomes and evidence based practice committee systematic review. J Pediatr Surg. 50(10):1783-90, 2015

PSU Volume 46 NO 04 APRIL 2016

Saline Hydrostatic Intussusception Reduction

An intussusception is an invagination of the proximal bowel into the lumen of the distal bowel. Most (>90%) intussusception in children are ileocolic, with the least of them being either ileoileal or colocolic. Swollen Peyer's patches, enlarged lymph nodes, polyps, Meckel's diverticulum and duplication cysts are the most common etiological factors associated with intussusceptions. They occur usually in children between the ages of six months to three years demonstrating signs/symptoms such as colicky pain, bilious vomiting, abdominal distension, and currant jelly stools. The diagnosis of intussusception can be made using an abdominal ultrasound when demonstrating an oval pseudokidney mass lesion with central echoes, a sonolucent target lesion, various layers and concentric rings, fluid, enlarged lymph nodes or other soft tissue or cystic mass suggestive of a pathological lead point. Ultrasonographically guided hydrostatic reduction with the use of normal warm saline is believed to be one of the most promising method for the non-surgical treatment of pediatric intussusception. It has been found to be safe, simple, effective, economical, and a less time-consuming procedure coupled with fewer complications, no radiation hazard and a minimal hospital stay. It is also associated with less morbidity since there is no incidence of a pseudo-obstruction as is seen in the fluoroscopic guided procedure, there is no fluctuation in the intracolonic pressure and there is minimal chance for chemical peritonitis as it occurs when using other contrast material. Risk factors for failure of reduction include localization (left colon), bloody stools, free peritoneal fluid and fluid trapped in the intussusceptum. Contraindications for hydrostatic reduction include absent or scattered vascularity within the lesion in color Doppler studies, evident signs of perforation or peritonitis, ascites, multiple intussusception with a pathological lead point or shock. The reduction success rate using saline hydrostatic reduction monitored by ultrasound is above 80%.

1- Shehata S, El Kholi N, Sultan A, El Sahwi E: Hydrostatic reduction of intussusception: barium, air, or saline? Pediatr Surg Int. 16(5-6):380-2, 2000
2- Nayak D, Jagdish S: Ultrasound guided hydrostatic reduction of intussusception in children by saline  enema: our experience. Indian J Surg. 70(1):8-13, 2008
3- Mensah Y, Glover-Addy H, Etwire V, Appeadu-Mensah W, Twum M: Ultrasound guided hydrostatic reduction of intussusception in children at Korle Bu Teaching Hospital: an initial experience. Ghana Med J. 45(3):128-31, 2011
4- Digant SM, Rucha S, Eke D: Ultrasound guided reduction of an ileocolic intussusception by a hydrostatic method by using normal saline enema in paediatric patients: a study of 30 cases. J Clin Diagn Res. 6(10):1722-5, 2012
5- He N, Zhang S, Ye X, Zhu X, Zhao Z, Sui X: Risk factors associated with failed sonographically guided saline hydrostatic intussusception reduction in children. J Ultrasound Med. 33(9):1669-75, 2014
6- Flaum V, Schneider A, Gomes Ferreira C, et al: Twenty years' experience for reduction of ileocolic intussusceptions by saline enema under sonography control. J Pediatr Surg. 51(1):179-82, 2016

Perforated Peptic Ulcer Disease

Peptic ulcer disease (PUD) is uncommon in children and rarely suspected as an etiology of abdominal symptoms until the child develops a complication such as upper gastrointestinal bleeding, obstruction or perforation. Peptic ulcer disease is classified as gastric or duodenal depending on location. Children less than 10 years of age predominantly have duodenal peptic ulcers, while above that age gastric ulcer predominates. Most duodenal peptic ulcer disease is associated with Helicobacter Pylori infection of the gastric antral mucosa. Other causes of PUD results from medications (aspirin, NSAID, steroids) and severe stress (burns, head trauma, Crohn's disease). Peptic ulcer perforation occurs in almost 12% of children with peptic ulcer disease. Children with PUD perforation present with an acute sudden onset painful abdomen, vomiting, nocturnal awakening and generalized peritonitis with board like abdomen. Almost 90% of children will show free air under the diaphragm in plain abdominal films. CT-Scans are more sensitive in establishing the diagnosis of free or contained perforation. Laparoscopy can be utilized for diagnosis and management of perforated PUD. Surgical management of perforated PUD depends on the severity of the disease and extent of perforation. Most small perforations can be managed with laparoscopic simple closure using an omental patch. Laparoscopic omental patch is the easiest, quickest and safest treatment. In complex or large perforated duodenal ulcers open definitive procedure such as vagotomy or gastric resections may be required due to larger spillage of GI content, more sepsis and instability. Postoperatively these patients will require eradication of Helicobacter pylori if breath test or biopsy is positive along with proton pump inhibitors. The rate of PUD recurrence is very high in untreated H. Pylori infection.  

1- Hua MC, Kong MS, Lai MW, Luo CC: Perforated peptic ulcer in children: a 20-year experience.J Pediatr Gastroenterol Nutr. 45(1):71-4, 2007
2- Yadav RP, Agrawal CS, Gupta RK, Rajbansi S, Bajracharya A, Adhikary S: Perforated duodenal ulcer in a young child: an uncommon condition. JNMA J Nepal Med Assoc. 48(174):165-7, 2009
3- Coppolino F, Gatta G, Di Grezia G, Reginelli A, Iacobellis F, Vallone G, Giganti M, Genovese E: Gastrointestinal perforation: ultrasonographic diagnosis. Crit Ultrasound J. 5 Suppl 1:S4, 2013
4- Schwartz S, Edden Y, Orkin B, Erlichman M: Perforated peptic ulcer in an adolescent girl. Pediatr Emerg Care. 28(7):709-11, 2012
5- Yildiz T, Ilce HT, Ceran C, Ilce Z: Simple patch closure for perforated peptic ulcer in children followed by helicobacter pylori eradication. Pak J Med Sci. 30(3):493-6, 2014
6- Wong CW, Chung PH, Tam PK, Wong KK: Laparoscopic versus open operation for perforated peptic ulcer in pediatric patients: A 10-year experience. J Pediatr Surg. 50(12):2038-40, 2015

OEIS Complex

OEIS complex is a very rare group of malformations which includes omphalocele, exstrophy of cloaca, imperforate anus and spinal defect. This constellation has also been labeled exstrophy of the cloaca, exstrophia splanchnica, vesicointestinal fissure, and ectopic cloaca. It represents the most severe form of exstrophy-epispadia complex occurring at a rate of one in 200-400,000 live births. Most cases are sporadic with no obvious etiology. OEIS complex may be difficult to diagnose prenatally. Currently there is no clear distinction how OEIS complex occurs during development. OEIS complex can also be associated with spina bifida, genital abnormalities, renal malformations, symphysis pubis diastasis and limb abnormalities. Exstrophy of the cloaca includes the persistence and exstrophy of a common cloaca that receives ureters, ileum and a rudimentary hindgut. Terminal myelocystoceles constitute approximately 5% of skin-covered lumbosacral masses and are especially common in patients with cloacal exstrophy or the OEIS complex. Most patients with OEIS complex have a single umbilical artery. Associated cardiac defects are rare. Brain is normal and most patients have normal intelligence. Suspicion of OEIS complex prenatally occurs when the US reveals nonvisualization of the bladder, infra-umbilical anterior abdominal wall defect, omphalocele and myelomeningocele. Prenatal MRI is diagnostic of the complex malformation helping plan surgery and provide family/genetic counseling before birth. Management of OEIS complex requires and interdisciplinary surgical approach from pediatric surgery, pediatric urology, orthopedics and neurosurgery. The driving factor behind surgical correction is to prevent postrenal obstructive uropathy. Surgical treatment has resulted in improved survival and quality of life.

1- Ben-Neriah Z, Withers S, Thomas M, Toi A, Chong K, Pai A, Velscher L, Vero S, Keating S, Taylor G, Chitayat D: OEIS complex: prenatal ultrasound and autopsy findings. Ultrasound Obstet Gynecol. 29(2):170-7, 2007
2- Sawaya D, Gearhart JP: Gastrointestinal reconstruction and outcomes for patients with the OEIS complex. Semin Pediatr Surg. 20(2):123-5, 2011
3- Phillips TM, Salmasi AH, Stec A, Novak TE, Gearhart JP, Mathews RI: Urological outcomes in the omphalocele exstrophy imperforate anus spinal defects  (OEIS) complex: experience with 80 patients. J Pediatr Urol. 9(3):353-8, 2013
4-Mandrekar SR, Amoncar S, Banaulikar S, Sawant V, Pinto RG: Omphalocele, exstrophy of cloaca, imperforate anus and spinal defect (OEIS Complex) with overlapping features of body stalk anomaly (limb body wall complex). Indian J Hum Genet. 20(2):195-8, 2014
5- Santoro JD, Chao S, Hsieh MH, Lee HC: Multimodality Renal Failure in a Patient with OEIS Complex. AJP Rep. 5(2):e161-4, 2015
6- Allam ES, Shetty VS, Farmakis SG: Fetal and neonatal presentation of OEIS complex. J Pediatr Surg. 50(12):2155-8, 2015

PSU Volume 46 NO 05 MAY 2016

Congenital Pulmonary Airway Malformations

Congenital pulmonary airway malformations (CPAM) refer to an unusual lesion of the pulmonary airways which combines features of hamartoma malformation and dysplastic proliferation. CPAM includes cystic pulmonary airway malformations, bronchopulmonary sequestration, bronchogenic cysts, hybrid lesions and  lobar/segmental emphysema causing respiratory distress in 20-40% of affected babies in the postnatal period. The remaining cases continue asymptomatic or develop symptoms later in life such as chest infections. Most CPAM can be detected on the 20-week antenatal ultrasound increasing the diagnostic yield if MRI is utilized. Children with symptoms early in life are managed with surgery. The management of asymptomatic CPAM is a source of controversy in the literature. CPAM is classified 0 to IV. Type 0 is very rare described as acinar aplasia or agenesis and incompatible with life. Type I the most common is primarily macrocystic with large single or multiple cysts several centimeters in size. Type II is microcystic and associated with other anomalies. Type III appears more solid or with very small cysts similar to immature lungs without bronchi. Type IV originates from the acinus and present with small cysts on the periphery of the lung lobes. Once a cystic lesion is detected in antenatal ultrasound, the location, volume, size, macrocystic or microcystic classification and blood supply should be evaluated. CPAM volume to head circumference ratio (CVR) greater than 1.6 results in fetal demise in about 80% of cases without fetal intervention. CVR < 1.6 will often not continue to grow past the 28th week of gestation. The reasons used to remove asymptomatic lesions in the first year of life include the rate of empyema, abscess, recurrent pneumonia, air leak, pneumothorax and malignancy. Almost 25% of asymptomatic children show histologic evidence of infection. CPAM have a long-term risk of malignancy. Multiple courses of antenatal betamethasone for high-risk fetal CPAM often results in favorable short-term outcomes without the need for open fetal resection. 

1- Baird R, Puligandla PS, Laberge JM: Congenital lung malformations: informing best practice. Semin Pediatr Surg. 23(5):270-7, 2014
2- Derderian SC, Coleman AM, Jeanty C, Lim FY, Shaaban AM, Farrell JA, Hirose S, MacKenzie TC, Lee H: Favorable outcomes in high-risk congenital pulmonary airway malformations treated
with multiple courses of maternal betamethasone. J Pediatr Surg. 50(4):515-8, 2015
3- Gajewska-Knapik K, Impey L: Congenital lung lesions: Prenatal diagnosis and intervention. Semin Pediatr Surg. 24(4):156-9, 2015
4- Singh R, Davenport M: The argument for operative approach to asymptomatic lung lesions. Semin Pediatr Surg. 24(4):187-95, 2015
5- Macardle CA, Ehrenberg-Buchner S, Smith EA, Dillman JR, Mychaliska GB, Treadwell MC, Kunisaki SM: Surveillance of fetal lung lesions using the congenital pulmonary airway malformation volume ratio: Natural history and outcomes. Prenat Diagn. 2015 Dec 29
6- Durell J, Thakkar H, Gould S, Fowler D, Lakhoo K: Pathology of asymptomatic, prenatally diagnosed cystic lung malformations. J Pediatr Surg. 51(2):231-5, 2016

Cutaneous Schwannomas

Schwannoma is a benign, expansile tumor that originates from any nerve sheath in the body that contains Schwann cell. Schwann cells insulate normal nerve fibers and enhance propagation of nerve impulses. Schwannomas can occur anywhere in the body along the course of a nerve such as cranial nerves, spinal nerves or peripheral nerves.  As such most schwannomas appear intracranially, intraspinal or lying deep within soft-tissue. The most common schwannoma is the acoustic neuroma along the VIII cranial nerve. Cutaneous schwannomas present as a solitary deep-seated nodule in the deep dermis or subcutaneous tissue. Most cutaneous schwannomas are asymptomatic. Other times they may cause pain, tenderness or paresthesia depending on size, site and nerve involved. Males and females are equally affected. Histologically, schwannomas are encapsulated by perineurium and contain either cellular areas characterized by uniform spindle cell clustered in stack and arranged back to back, or a loose myxoid matrix. Immunohistochemistry of schwannomas reveals positive S100 and collagen type 4, and capsule is positive for epithelial membrane antigen. Schwannomas enlarge slowly and follow a benign course with very rare cases of malignant transformation. The preoperative diagnosis of a cutaneous schwannoma is seldom done as the nodule is usually confused with an epidermal, trichilemmal or dermoid cyst. Among the other types of schwannomas, plexiform variety constitute 5% of the presentation associated with neurofibromatosis. Few reports of malignant transformation in schwannomas have been reported in the literature. Management of schwannomas is surgical excision which is usually curative. Local recurrence can occur and long-term follow up is needed.  

1- Agaram NP, Prakash S, Antonescu CR: Deep-seated plexiform schwannoma: a pathologic study of 16 cases and comparative  analysis with the superficial variety. Am J Surg Pathol. 29(8):1042-8, 2005
2- Attia EA, Yassin M, Lasheen MA, Salem SA, Khafagy NH: Multiple isolated cutaneous plexiform schwannomas.  Indian J Dermatol Venereol Leprol. 77(5):594-6, 2011
3- Elwood H, Taube J: Dermal and Subcutaneous Plexiform Soft Tissue Neoplasms. Surg Pathol Clin. 4(3):819-42, 2011
4- Noh S, Do JE, Park JM, Jee H, Oh SH: Cutaneous schwannoma presented as a pedunculated protruding mass. Ann Dermatol. 23(Suppl 2):S264-6, 2011
5- Mohan Kh, Manjunath H: Cutaneous schwannoma masquerading as trichilemmal cyst over scalp in a young male. Indian J Dermatol. 58(5):407, 2013
6- Kacerovska D, Michal M, Kazakov DV: Hybrid Epithelioid Schwannoma/Perineurioma. Am J Dermatopathol. 2016 Feb 9.

Enteral Refeeding

Many neonates undergo intestinal resections for a variety of conditions losing a significant segment of bowel including the construction of proximal enterotomies with distal mucous fistulas. They rely on parenteral nutrition for a significant period of time after surgery. Parenteral nutrition is associated with a significant risk of catheter-related blood stream infection, thrombosis, and neonatal cholestasis. The absence of food in the GI tract produces mucosal and villous atrophy with reduction of enzymes necessary for digestion and substrate absorption. Promoting early enteral nutrition is beneficial even in the face of a proximal enterostomy. The presence of a proximal enterostomy can produce high stomal losses with associated fluid and electrolytes imbalances, metabolic acidosis and impaired absorption of nutrition. Refeeding of stomal losses into the distal mucous fistula  has been used to minimized fluid and electrolytes losses as well as dependence on parenteral nutrition. Mucous fistula refeeding (MFR) stimulates mucosal growth and intestinal adaptation preventing atrophy of the distal gut. Enteral refeeding requires substantial nursing expertise, time and commitment to the process. MFR has also been associated with distal perforation with bacterial overgrowth in the stomal output if there is a delay between collection and refeeding of the stoma effluent. Fluoroscopy can be used to insert the distal refeeding tube to be utilized taking care to determine if there is thin intestinal wall propene to perforation or distal strictures in the case of NEC babies. Neonates with enteral refeeding have a better gain in body weight than those without it, regardless of the gestational age. Only the MFR procedure and birth weight were significant independent predictors of a good weight gain. Babies using MFR have a high rate of central venous catheter removal because of nutritional improvement. The longer the distal bowel to be fed the better the rate of body weight gain.  

1- Hay WW Jr: Strategies for feeding the preterm infant. Neonatology. 94(4):245-54, 2008
2- Morgan J, Bombell S, McGuire W: Early trophic feeding versus enteral fasting for very preterm or very low birth weight infants. Cochrane Database Syst Rev. Mar 28;3:CD000504, 2013
3- Haddock CA, Stanger JD, Albersheim SG, Casey LM, Butterworth SA: Mucous fistula refeeding in neonates with enterostomies. J Pediatr Surg. 50(5):779-82, 2015
4- Wong KK, Lan LC, Lin SC, Chan AW, Tam PK: Mucous fistula refeeding in premature neonates with enterostomies. J Pediatr Gastroenterol Nutr. 39(1):43-5, 2004
5- Al-Harbi K, Walton JM, Gardner V, Chessell L, Fitzgerald PG: Mucous fistula refeeding in neonates with short bowel syndrome. J Pediatr Surg. 34(7):1100-3, 1999
6- Koike Y, Uchida K, Nagano Y, Matsushita K, Otake K, Inoue M, Kusunoki M: Enteral refeeding is useful for promoting growth in neonates with enterostomy before stoma closure. J Pediatr Surg. 51(3):390-4, 2016

PSU Volume 46 NO 06 JUNE 2016

Retained Appendicolith

Appendicoliths are formed by calcium phosphate, inorganic salts and fecal debris layered and lodged within the appendix lumen that may cause obstruction, inflammation and perforation of the organ. They are present in up to 30% of children with appendicitis. When an appendicolith is found extraluminally, it is pathognomonic for perforation of the appendix. Rarely an appendicolith may be retained from a perforated appendix or dropped during appendectomy. Retained appendicolith is a delayed complication associated after previous removal of the appendix using the laparoscopic or open surgical technique. Dropped or retained appendicolith has been reported to occur more frequently in the setting of laparoscopic surgery compared to open appendectomy due to failure to recognize and extract the appendicolith during the procedure Appendicolith retained in the peritoneal cavity after an appendectomy may serve as a nidus of infection and lead to abscess formation days to months after surgery. The median time to presentation of the abscess is 1.5 weeks with a mean of two months, though a few reports demonstrate presentation to occur several years later. Children present most commonly with focal abdominal pain, fever and leukocytosis. The most common finding on contrast-enhanced abdominal CT scans is an abscess with one or more subcentimeter foci of high attenuation adjacent to the cecum or the Morrison pouch. Retained appendicolith has been reported in different sites including the pelvis, gluteal region, hepatorenal pouch (Morrison) and subhepatic region. Every effort must be made to identify and retrieve appendicoliths at the initial appendectomy. In cases of retained appendicoliths the definitive management is surgical removal of the appendicolith as failure to do so may result in recurrent intra-abdominal abscesses, wound infection and occasionally fistula formation. Retrieval of the retained appendicolith can be performed open, laparoscopically and in a few instances percutaneously.

1- Lee SW, Lim JS, Hyung WJ, Park MS, Kim MJ, Cha SW, Yoo HS, Kim KW: Laparoscopic ultrasonography for localization of a retained appendicolith after appendectomy. J Ultrasound Med. 25(10):1361-3, 2006
2- Lapus RM, Baker MD: An uncommon late complication of appendicitis. Pediatr Emerg Care. 26(10):757-8, 2010
3- Maatouk M, Bunni J, Schuijtvlot M: Perihepatic abscess secondary to retained appendicolith: A rare complication managed laparoscopically. J Surg Case Rep. 2011(1):6-9, 2011
4- Whalley HJ, Remoundos DD, Webster J, Silva MA: Shortness of breath, fever and abdominal pain in a 21-year-old student. BMJ Case Rep. 14;2013, 2013
5- Black MT, Ha BY, Kang YS, Garland AM: Perihepatic abscess caused by dropped Appendicolith following laparoscopic appendectomy: sonographic findings.  J Clin Ultrasound. 41(6):366-9, 2013
6- Betancourt SL, Palacio D, Bisset GS 3rd: The 'wandering appendicolith'. Pediatr Radiol. 45(7):1091-4, 2015


Melanonychia striata or longitudinal refers to deposition of pigment in the nail plate from increased pigment production within the nail matrix. Melanonychia is a benign condition found rarely in children and adults. The deposition can involve a single longitudinal line or all the nail. The nail plate is not normally pigment, irrespective of race. Melanonychia is mainly due to melanin pigment produced by activating or proliferating melanocytes in the matrix. The problem arises in the differential diagnosis which includes lentigo, subungual nevus, atypical melanocytic hyperplasia or melanoma of the nail matrix in which case a biopsy is in order. Clinical features of melanonychia that increase concern whether this is a melanoma includes pigment bands broader that 3 mm, changing pigmentation or shape, associated nail dystrophy, Hutchinson sign, bands that are not homogenous in color, blurred lateral borders, irregular lines that are not parallel on dermoscopy and rapid evolution. History of such changes is an indication for nail matrix biopsy. Should the biopsy show high melanocyte count, pagetoid spread, presence of nuclear atypia and/or predominance of single units of melanocytes this would then favor the diagnosis of melanoma of the nail matrix. Melanonychia striata does not progress to melanoma, hence the majority of cases of melanonychia can be managed conservatively without needing to remove the nail and affected matrix (nail avulsion or tangential excision). Follow up is needed in cases that are not backup by biopsy using dermatoscopic discrimination index evaluating changes in color variegation over time. The reluctance of biopsy is the high incidence of developing nail dystrophy as complication of the procedure.

1- Murata Y, Kumano K: Dots and lines: a dermoscopic sign of regression of longitudinal melanonychia in  children. Cutis. 90(6):293-6, 301, 2012
2- Richert B, Andre J: Nail disorders in children: diagnosis and management. Am J Clin Dermatol. 12(2):101-12, 2011
3- Tosti A, Piraccini BM, Cagalli A, Haneke E: In situ melanoma of the nail unit in children: report of two cases in fair-skinned Caucasian children. Pediatr Dermatol. 29(1):79-83, 2012
4- Richert B, Theunis A, Norrenberg S, Andre J: Tangential excision of pigmented nail matrix lesions responsible for longitudinal melanonychia: evaluation of the technique on a series of 30 patients. J Am Acad Dermatol. 69(1):96-104, 2013
5- Cooper C, Arva NC, Lee C, Yalamos O, Obregon R, Sholl LM, Wagner A, Shen L, Guitart J, Gerami P: A clinical, histopathologic, and outcome study of melanonychia striata in childhood.  J Am Acad Dermatol. 72(5):773-9, 2015
6- Koga H, Yoshikawa S, Shinohara T, Le Gal FA, Cortes B, Saida T, Sota T: Long-term Follow-up of Longitudinal Melanonychia in Children and Adolescents Using an Objective Discrimination Index. Acta Derm Venereol. Jan 25, 2016

Central Venous Catheter Repair

Central venous catheters (CVC) are essential for providing prolonged enteral nutrition, chemotherapy and medications in critically and chronically ill pediatric patients. In cases of gut failure or absence they are the primordial way to provide nutrition for extended period of time. They create the venue for outpatient management for many ailments in children. As foreign body inserted into the body they can get infected, thrombosed, dislodge or suffer mechanical complications such as breakage needing frequent change in the life of patients using them as life supporting. Implanted CVC should be avoided in children with neutropenia (absolute neutrophil count less than 0.5 x 109/L) due to the increase incidence of bloodstream infection. In pediatric patients the rate of breakage of the external portion of the catheter is one per 1000 catheter-days. When breakage occurs the surgeon has the choice of repairing the catheter or replace it depending on surgical risk and patent available veins. Repairs are performed using the kits sold by the manufacturers of most CVC. Repair can be done using glue after cutting the break site and placing a plastic sheath slide over the new union site to splint the repair, or without glue with an extension catheter with adaptor or connector piece lock into place into the original catheter. A break in the CVC that breaches the lumen is expected to compromise its sterility. Repair and retention of that catheter could predispose the patient to a 2-4 fold higher risk of bloodstream infection in the 30 days following repair. Age, underlying diagnosis, immunocompromised state, parenteral nutrition, catheter type or repair type modified the effect of catheter break and repair on the risk of bacteremia and infection. To stir further the controversy, in gut failure children repairing CVC increases line longevity and preserves the use of limited vascular access sites avoiding early transplants due to absent vascular access. Also, in gut failure cases repairing CVC was not associated with an increase rate of line infection.

1- Moukarzel AA, Haddad I, Ament ME, Buchman AL, Reyen L, Maggioni A, Baron HI, Vargas J: 230 patient years of experience with home long-term parenteral nutrition in childhood: natural history and life of central venous catheters. J Pediatr Surg. 1994 29(10):1323-7, 1994
2- Elihu A, Gollin G: Complications of implanted central venous catheters in neutropenic children. Am Surg. 73(10):1079-82 2007
3- Milbrandt K, Beaudry P, Anderson R, Jones S, Giacomantonio M, Sigalet D: A multiinstitutional review of central venous line complications: retained intravascular fragments. J Pediatr Surg. 44(5):972-6, 2009
4- Abu-El-Haija M, Schultz J, Rahhal RM: Effects of 70% ethanol locks on rates of central line infection, thrombosis, breakage, and replacement in pediatric intestinal failure.  J Pediatr Gastroenterol Nutr. 58(6):703-8, 2014
5- Lundgren IS, Zhou C, Malone FR, McAfee NG, Gantt S, Zerr DM: Central venous catheter repair is associated with an increased risk of bacteremia and central line-associated bloodstream infection in pediatric patients. Pediatr Infect Dis J. 31(4):337-40, 2012
6- McNiven C, Switzer N, Wood M, Persad R, Hancock M, Forgie S,  Dicken BJ: Central venous catheter repair is not associated with an increased risk of central line infection or colonization in intestinal failure pediatric patients. J Pediatr Surg. 51(3):395-7, 2016

Journal Club