PSU Volume 48 No 01 JANUARY 2017

Retroperitoneal Sarcomas

Soft tissue sarcomas are a heterogenous group of rare tumors arising from embryonic mesoderm. Almost 15% of such sarcomas arise in the retroperitoneum. Rhabdomyosarcoma and fibrosarcoma are the two most common histologic variants in the retroperitoneum. The prognosis for patients with retroperitoneal sarcomas (RPS) is relatively poor characterized by late locoregional recurrence as principal cause of death. In the retroperitoneum tumor growth has a large capacity before causing overt symptoms reaching enormous size and invading adjacent vital vascular structures. At diagnosis RPS are the largest tumors found in the human body. Even with large size RPS rarely metastasize. The best potential curative treatment (a survival factor) is macroscopically complete, margin-negative gross surgical resection. The size and complexity of RPS tumors result in microscopically residual disease after surgery needing the use of adjuvant chemo- and radiotherapy. Rhabdomyosarcoma (RMS) is the most common soft tissue sarcoma arising in the retroperitoneum in children. Retroperitoneal RMS are quite large and seen at CT as a bulky mass with heterogenous attenuation equal to or slightly less than muscle. Areas of attenuation representing necrosis are common and calcifications are rare. The precise origin of the tumor is often difficult to determine because of infiltration of adjacent organs. Retroperitoneal and inguinal lymph node enlargement and bone and lung metastasis may be seen. RMS is more responsive to chemo- and radiotherapy in children than adults. Tumor histology and responsiveness to neoadjuvant therapy influence resectability. Debulking of RMS in combination with chemo- and radiotherapy induce tumor shrinkage and facilitate tumor resection improving survival. Children with low-grade tumors have better survival as compared to those with high-grade sarcomas. The efficacy of current chemotherapy is limited and there is a critical need to understand the molecular basis of sarcomas so that new drug therapies are developed.

1- Porter GA(1), Baxter NN, Pisters PW: Retroperitoneal sarcoma: a population-based analysis of epidemiology, surgery, and radiotherapy. Cancer. 106(7):1610-6, 2006
2- Pham TH(1), Iqbal CW, Zarroug AE, Donohue JH, Moir C: Retroperitoneal sarcomas in children: outcomes from an institution. J Pediatr Surg. 42(5):829-33, 2007
3- Xu Y, Wang J, Peng Y, Zeng J: CT characteristics of primary retroperitoneal neoplasms in children. European J of Radiology. 75: 321-328, 2010
4- Stucky CC(1), Wasif N, Ashman JB, Pockaj BA, Gunderson LL, Gray RJ: Excellent local control with preoperative radiation therapy, surgical resection, and intra-operative electron radiation therapy for retroperitoneal sarcoma.  J Surg Oncol. 109(8):798-803, 2014
5- Wolden SL(1), Lyden ER(2), Arndt CA(3), Hawkins DS(4), Anderson JR(5), Rodeberg DA(6), Morris CD(7), Donaldson SS(8): Local Control for Intermediate-Risk Rhabdomyosarcoma: Results From D9803 According to Histology, Group, Site, and Size: A Report From the Children's Oncology Group. Int J Radiat Oncol Biol Phys. 93(5):1071-6, 2015
6- Gladdy RA(1), Gupta A(2), Catton CN(3): Retroperitoneal Sarcoma: Fact, Opinion, and Controversy. Surg Oncol Clin N Am. 25(4):697-711, 2016

Renal Cell Carcinoma

Renal cell carcinoma (RCC) is an uncommon malignant tumor arising from an epithelial cell of the renal tubules accounting for 3% of all pediatric renal tumors. Median age is between 8 and 17 years with no gender predominance. Underlying associated conditions includes tuberous sclerosis and prior chemotherapy.  Most RCC  presents with symptoms such as flank pain, hematuria and abdominal mass with few cases diagnosed after incidental radiology studies, usually ultrasound. Children present with higher stage, higher grade and larger tumors when compared with older patients. Diagnosis is confirmed with CT-Scan and MRI. 30% of pediatric RCC presents with metastatic disease such as lymphadenopathy, vascular involvement, local and distant metastasis to liver, contralateral kidney or lungs. Differential diagnosis includes nephroblastoma. Calcifications are a single radiologic feature associated with 50% of RCC. Pathologic subtypes of RCC include the papillary histology most commonly (30-80%) followed by relative dearth of clear cell type (17-50%). In children RCC demonstrates translocation in the Xp11.2 (TFE3 gene) most commonly followed by the 6p21 loci (TFEB gene). Translocation tumors tend to have rather indolent disease with a good outcome even in the presence of advance disease. Children with Von Hippel Lindau syndrome typically develop clear cell RCC at a young age which can be multifocal or bilateral. Neuroblastoma survivors have a 300-fold increase risk of developing RCC. Surgical excision is the mainstay treatment of RCC and a significant prognostic factor. Radical nephrectomy is the most commonly used surgical procedure. Partial nephrectomy is performed in tumors less than 4 cm, location amenable to partial resection and Robson stage 1 or 2 lesions with and excellent five year survival. Children with associated syndromes and RCC should also under partial nephrectomy since they will require repeated resections. Laparoscopic nephrectomy has been proved equally effective to open surgery in RCC when the tumor does not cross the midline. Long-term survival of RCC is affected by tumor size, lymph node status and pathologic stage.    

1- Liu JB, Lu ZB, Xiao XM: Laparoscopic Radical Nephrectomy of Wilms' Tumor and Renal Cancer in Children: Preliminary Experience from a Two-Center Study in China. J Laparoendosc Adv Surg Tech A. 25(6):516-21, 2015
2- Akhavan A, Richards M, Shnorhavorian M, Goldin A, Gow K, Merguerian PA: Renal cell carcinoma in children, adolescents and young adults: a National Cancer Database study. J Urol. 193(4):1336-41, 2015
3- Rialon KL, Gulack BC, Englum BR, Routh JC, Rice HE: Factors impacting survival in children with renal cell carcinoma. J Pediatr Surg. 50(6):1014-8, 2015
4- Canning DA: Re: Comparison between Laparoscopic and Open Radical Nephrectomy for the Treatment of Primary Renal Tumors in Children: Single-Center Experience over a 5-Year Period.   J Urol. 194(2):517, 2015
5- Abdellah A, Selma K, Elamin M, Asmae T, Lamia R, Abderrahmane M, Sanaa el M, Hanan E, Tayeb K, Noureddine B: Renal cell carcinoma in children: case report and literature review. Pan Afr Med J. 29;20:84, 2015
6- Young EE, Brown CT, Merguerian PA, Akhavan A: Pediatric and adolescent renal cell carcinoma. Urol Oncol. 34(1):42-9, 2016

Incisional Hernias

Incisional hernia (IH) is a frequent postoperative complication after abdominal surgery in children and adults. Incisional hernia occurs with greater incidence following open surgical procedures than with laparoscopic procedures. Emergency neonatal laparotomies are the mot common primary surgery associated with incisional hernias, with necrotizing enterocolitis comprising the major group. IH presents clinically as a reducible bulging in the scar area. Almost one-third of the patients who had an IH were unaware of the presence of the hernia. Ultrasound and CT-Scans increase the rate of detection of incisional hernias. Risk factors associated in the development of IH in children include age less than six months, wound infection, median incisions and emergency procedure. Most IH will developed in the next two years after the original abdominal procedure. Vertical incisions have a greater incidence of hernia development than transverse abdominal procedures in children. Guidelines to avoid incisional hernias include avoiding vertical incisions and closure using an absorbable monofilament suture in a single layer fascia closure technique without separate closure of the peritoneum. For laparoscopic surgery recommendations of closing the port defect whenever feasible, especially those of 10 mm. Indications for repair of incisional hernia should include symptoms of pain, limitation in daily activity and evident enlargement of the hernia defect. Methods of repair include primary closure whenever possible or mesh repair using open or laparoscopic technique. The most common group of pediatric patient who underwent an IH repair were those following closures of stomas.

1- Davies M, Davies C, Morris-Stiff G, Shute K: Emergency presentation of abdominal hernias: outcome and reasons for delay in treatment - a prospective study. Ann R Coll Surg Engl. 89(1):47-50, 2007
2- Hussain A, Mahmood H, Singhal T, Balakrishnan S, Nicholls J, El-Hasani S: Long-term study of port-site incisional hernia after laparoscopic procedures. JSLS. 13(3):346-9, 2009
3- Kenchadze G, Pipia I, Demetrashvili Z, et al: Incisional Hernia: Plastic Aspects, Component Separation, Technical Details & Pediatrics.  Hernia. 19 Suppl 1:S187-94, 2015
4- Sharp SP, Francis JK, Valerian BT, Canete JJ, Chismark AD, Lee EC: Incidence of Ostomy Site Incisional Hernias after Stoma Closure. Am Surg. 81(12):1244-8, 2015
5- Mullassery D, Pedersen A, Robb A, Smith N: Incisional hernia in pediatric surgery - experience at a single UK tertiary centre. J Pediatr Surg. 51(11):1791-1794, 2016

PSU Volume 48 NO 02 FEBRUARY 2017

Barrett Metaplasia in Esophageal Atresia

The prevalence of gastroesophageal reflux is increased significantly in children born and managed for esophageal atresia (EA). Chronic untreated gastroesophageal reflux can lead to malnutrition, esophagitis, esophageal strictures and intestinal metaplasia of the esophagus epithelium known as Barrett esophagus (BE). BE is defined as a change in the esophageal epithelium of any length that can be recognized at endoscopy and is confirmed to have intestinal columnar metaplasia by biopsy. The gastric type of metaplasia resembles the epithelium found in the gastric fundus and cardia, whereas the intestinal type of metaplasia (which is also called specialized columnar epithelium) has goblet cells as seen in intestinal mucosa. BE associated with intestinal metaplasia is a well-known risk factor for development of adenocarcinoma of the esophagus with a 30- to 125-fold increase compared with the general population. Long term results have found that heartburn, dysphagia and retrosternal pain, symptoms of gastroesophageal reflux might be present in almost one-third of all children repaired of EA as infant. Dysphagia occurs due to impaired motility, esophagitis, and anastomotic or peptic structure formation. BE is rare in children without neurodevelopmental delay or tracheoesophageal anomalies like esophageal atresia. Duration of symptoms and/or age related effects are important risk factors for BE development. The lag time  to developing metaplasia from the time of initial surgical correction is about ten years. Endoscopy and biopsies are the best way of detecting such mucosal changes. Recently prevalence rates of 42% for gastric metaplasia and 1% for intestinal metaplasia has been found in adolescent and young adults with repaired EA. Characteristics of these patients include peptic esophagitis, previous multiple antireflux surgery, type I atresia and esophageal dilatation. Nissen fundoplication was found not to prevent BE in EA patients. Systematic upper GI endoscopy and multistaged biopsies should be performed before the transition to adulthood in all patients with EA, even if asymptomatic. If no BE is found, endoscopy should be repeated every five to 10 years through adulthood. 

1- Deurloo JA, Ekkelkamp S, Bartelsman JF, Ten Kate FJ, Schoorl M, Heij HA, Aronson DC: Gastroesophageal reflux: prevalence in adults older than 28 years after correction of esophageal atresia. Ann Surg. 238(5):686-9, 2003
2- Deurloo JA, Ekkelkamp S, Taminiau JA, Kneepkens CM, ten Kate FW, Bartelsman JF, Legemate DA, Aronson DC: Esophagitis and Barrett esophagus after correction of esophageal atresia. J Pediatr Surg. 40(8):1227-31, 2005
3- Burjonrappa SC, Youssef S, St-Vil D: What is the incidence of Barrett's and gastric metaplasia in esophageal atresia/tracheoesophageal fistula (EA/TEF) patients? Eur J Pediatr Surg. 21(1):25-9, 2011
4- Nguyen DM, El-Serag HB, Shub M, Integlia M, Henderson L, Richardson P, Fairly K, Gilger MA: Barrett's esophagus in children and adolescents without neurodevelopmental or tracheoesophageal abnormalities: a prospective study. Gastrointest Endosc. 73(5):875-80, 2011
5-  Maynard S, Bouin M: Follow-up of adult patients with repaired esophageal atresia: how, when, and for how long? Dis Esophagus. 26(4):422-4, 2013
6- Schneider A, Gottrand F, Bellaiche M, et al: Prevalence of Barrett Esophagus in Adolescent and Young Adults with Esophageal Atresia. Ann Surg 264(12): 1004-1008, 2016

Intrapericardial Teratoma

Tumors of the heart and pericardium are rare causing a variety of cardiac and systemic symptoms depending on the size and anatomic location. Growth rate, friability and ability to invasiveness can determine clinical features and outcome. Intrapericardial teratoma is a very rare congenital tumor which can be diagnosed in-utero or soon after birth due to its association with massive pericardial effusion. Intrapericardial teratoma is a germ cell origin tumor composed of the three primitive germ layers, namely endoderm (gastric and intestinal mucosa), ectoderm (neuroglia) and mesoderm (bone, cartilage, fatty or fibrous tissue) arising from the pericardium. Patient age ranges from intrauterine life to adulthood with most cases occurring in infants. More than 75% of intrapericardial teratomas occur in children under the age of fifteen. Main clinical symptoms include respiratory distress, pericardial tamponade and cyanosis. Most are mature type teratomas followed by immature cases. They can be diagnosed intrauterine using prenatal ultrasound with findings of a large pericardial effusion and intrapericardial multilobulated and cystic mass with calcifications. Diagnosis is confirmed using MRI and fetal echocardiogram. In cases of fetal cardiac tamponade or hydrops intrauterine pericardiocentesis can be performed to permit near full-term birth. Intrapericardial teratomas are usually located in the right side of the heart causing displacement and left-side rotation. The arterial supply is from a pedicle to one of the great vessels or directly from the aorta.  Surgical excision is the only effective management for intrapericardial teratoma. Since most tumors are benign the prognosis is usually good after resection. The presence of immature neuroepithelium carries a poor prognosis needing adjuvant radio- and chemotherapy. Surgical resection of the teratoma in the fetus through EXIT strategy or open fetal surgery is feasible if the tumor is growing fast and causing significant hemodynamic changes including hydrops or impending death.

1- Molina-Mora MJ, Picazo-Antolin B, Cuenca-Peira V, Miguel Gil-Jaurena J, Zabala-Arguelles JI: Foetal intrapericardial teratoma. Eur J Echocardiogr. 12(7):513, 2011
2- Oto O, GazeloÄŸlu M, Kir M, Metin K, Cakmaka H, Albayrak G, Koa A: Intrapericardial teratoma in a newborn: a case report. Turk J Pediatr. 54(1):71-3, 2012
3- Milovanovic V, Lukac M, Krstic Z: Intrapericardial immature teratoma in a newborn: a case report. Cardiol Young. 24(1):164-6, 2014
4- Malay J, Madhavi N, Satyavani A, Nishanth P, Manikyamba D: Intrapericardial immature teratoma with successful treatment in a neonate. Indian J Pediatr. 81(10):1099-1101, 2014
5- Singh V, Kakkar S, Arora A, Garg A, Harjai MM: A rare case of intra-pericardial teratoma presenting as a mediastinal mass in an infant. Med J Armed Forces India. 71(Suppl 1):S49-51, 2015
6- Rychik J, Khalek N, Gaynor JW, Johnson MP, Adzick NS, Flake AW, Hedrick HL: Fetal intrapericardial teratoma: natural history and management including successful in utero surgery. Am J Obstet Gynecol. 215(6):780, 2016

Tubo-ovarian Abscess

Tube-ovarian abscess (TOA) is a well known complication of pelvic inflammatory disease (PID) in young sexually-active women during reproductive years, including adolescents. TOA is an ascending infection from the cervix and/or vagina through the uterus to the fallopian tubes and ovaries. The infection is usually the result of sexually transmitted disease or after instrumentation of the female genital tract. Patients with TOA usually present with low abdominal/pelvic  pain, vomiting and fever. Pelvic examination shows adnexal mass or tenderness. White blood cell count is elevated. The diagnosis is made with the help of US, CT-Scan or MRI. In occasion the diagnosis cannot be separated from symptoms of appendicitis. Appropriate management of PID complicated with a tubo-ovarian abscess requires prompt initiation of empiric broad spectrum antibiotics effective against both Neisseria gonorrhea and Chlamydia trachomatis. In the majority of patient antibiotics is all the treatment that is needed. At least 24 hours of inpatient observation is recommended during therapy. It is estimated that almost 40% of women with TOA fail to respond within 48-72 hours of therapy needing drainage of the abscess either percutaneously or via laparoscopic approach. PID and TOA are extremely rare in non-sexually active or amenorrheic adolescent females. The etiology in such cases includes inflammatory bowel disease with hematogenous seeding of bacteria, recurrent urinary tract infection with urinary vaginal reflux, poor hygiene, obesity with vulvar adiposity and müllerian abnormalities. The organism most commonly identified in such cases of virginal adolescents is Escherichia Coli, alpha hemolytic streptococcus and Pasteurella multocida. Though most cases of TOA are due to PID, laparoscopy can elucidate the correct diagnosis in atypical cases such as virgins, postmenopausal or those that do not respond to antibiotherapy.

1- Goh WC, Beh ST, Chern B, Yap LK: A three year review on surgical treatment of tubo-ovarian abscess. Med J Malaysia. 57(3):292-7, 2002
2- Mollen CJ, Pletcher JR, Bellah RD, Lavelle JM: Prevalence of tubo-ovarian abscess in adolescents diagnosed with pelvic inflammatory disease in a pediatric emergency department. Pediatr Emerg Care. 22(9):621-5, 2006
3- Jeong WK, Kim Y, Song SY: Tubo-ovarian abscess: CT and pathological correlation. Clin Imaging. 31(6):414-8, 2007
4- Goodwin K, Fleming N, Dumont T: Tubo-ovarian abscess in virginal adolescent females: a case report and review of the literature.  J Pediatr Adolesc Gynecol. 26(4):e99-102, 2013
5- Kielly M, Jamieson MA: Pelvic inflammatory disease in virginal adolescent females without tubo-ovarian abscess. J Pediatr Adolesc Gynecol. 27(1):e5-7, 2014
6- Sordia-Hernandez LH, Serrano Castro LG(2), Sordia-Pineyro MO, Morales Martinez A, Sepulveda Orozco MC, Guerrero-Gonzalez G: Comparative study of the clinical features of patients with a tubo-ovarian abscess and patients with severe pelvic inflammatory disease. Int J Gynaecol Obstet. 132(1):17-9, 2016

PSU Volume 48 No 03 MARCH 2017

Petersen Hernia

Petersen hernia (PH) is a specific type of internal hernia where the small bowel migrates into the space between the caudal surface of the transverse mesocolon and the mesentery of the gastrojejunostomy limbs when either open or laparoscopic Roux-en-Y gastric bypass are performed for morbid obesity and biliopancreatic diversion. Clinical presentation is characterized by nonspecific symptoms of bowel obstruction such as postprandial abdominal pain, nausea and vomiting leading to delayed diagnosis and producing small bowel ischemia and even death. Some patients may have recurrent transient herniation and intermittent abdominal pain. The Petersen's space was initially described in 1900 as a space between the Roux limb and the transverse mesocolon formed after gastrectomy with Roux-en-Y reconstruction. Body weight loss is considered to be a risk factor for an internal hernia to develop. A greater loss of weight such as it occurs in bariatric surgery can induce an increase in the size of Petersen defect increasing the risk of an internal hernia. Antecolic reconstruction procedures may tend to specifically lead to Petersen hernia. Three types of Petersen hernia have been described: Type A involves the alimentary (Roux) limb, Type B involves the bilio-pancreatic limb and Type C involves the common channel.  The diagnosis of Petersen hernia is confirmed using oral and intravenous contrast CT-Scan with findings of whirl sign, target sign, small bowel obstruction, clustered loop, retraction of the mesentery, congestion of mesenteric fat and vessels, mushroom sign, hurricane sign, small bowel behind SMA and right-sided anastomosis. The whirl signs of mesenteric fat or vessels have been reported to be the best single predictors of Petersen hernia with sensitivity of 80% and specificity of 90%. A high index of suspicion should be maintained to diagnose a Petersen hernia. Management is surgical reduction of the internal hernia on an emergency basis.

1- de Bakker JK, van Namen YW, Bruin SC, de Brauw LM: Gastric bypass and abdominal pain: think of Petersen hernia. JSLS. 2012 Apr-Jun;16(2):311-3
2- Reiss JE, Garg VK: Bowel gangrene from strangulated Petersen's space hernia after gastric bypass. J Emerg Med. 2014 Feb;46(2):e31-4
3- Genser L, Carandina S, Soprani A: Petersen's internal hernia complicating a laparoscopic omega loop gastric bypass. Surg Obes Relat Dis. 2015 Sep-Oct;11(5):e33-4
4- Baba A, Yamazoe S, Dogru M, Okuyama Y, Mogami T, Kobashi Y, Nozawa Y, Aoyagi Y, Fujisaki H, Ogura M, Matsui J: Petersen hernia after open gastrectomy with Roux-en-Y reconstruction: a report of two cases and literature review. Springerplus. 2015 Dec 2;4:753.
5- Goh YL, Haworth A, Wilson J, Magee CJ: Life-threatening Petersen's hernia following open Beger's procedure. J Surg Case Rep. 2016 Mar 18;2016(3).
6- Kular KS, Prasad A, Ramana B, Baig S, Mahir Ozmen M, Valeti M,  Ribeiro R, De Luca M, Apers J, Mahawar KK: Petersen's hernia after mini (one anastomosis) gastric bypass. J Visc Surg. 2016 Aug;153(4):321

Multiple Intestinal Atresias

Bowel atresia is a common cause of surgical intestinal obstruction in newborns. Most bowel atresia occurs in the jejunoileum and are single in nature. Pathogenesis of an intestinal atresia is a late intrauterine vascular accident in the mesentery causing loss and discontinuity of a segment of bowel. Bowel atresias are classified into: Type I: an intraluminal diaphragm with seromuscular continuity. Type II: cord-like segment between the bowel blinds ends. Type IIIA: atresia with complete separation of blind ends and V-shaped mesenteric defect. Type IIIB: jejunal atresia with extensive mesenteric defect and distal ileum acquiring its blood supply entirely from a single ileocolic artery. The distal bowel coils itself around the vessel, giving the appearance of an "apple peel"deformity. Type IV: multiple atresias. Multiple intestinal atresias (MIA) are the rarest of them all often associated with absence of significant bowel length resulting in short bowel syndrome. Though multiple anastomosis may suggest a higher risk of complications such as stricture or leak, they are the most effective treatment to preserve the maximum intestinal length in children with MIA. The proximal bowel can be amenable to tapering or the serial transverse enteroplasty (STEP) procedure. This proximal dilated bowel can be taken out as a jejunostomy. To accomplish the multiple segmental anastomosis in the affected bowel a soft silastic catheter can be used as stent of each anastomosis and exteriorize as a proximal mucous fistula while the distal end of the catheter can be brought out of the abdomen through the appendix. Intestinal continuity can be established at a later operation. There is a syndrome of hereditary MIA with multiple intestinal atresias from the stomach to the rectum in association with immune deficiency. The most common congenital malformation associated with MIA is Meckel diverticulum.     

1- Chaet MS, Warner BW, Sheldon CA: Management of multiple jejunoileal atresias with an intraluminal SILASTIC stent.  J Pediatr Surg. 29(12):1604-6, 1994
2- Lambrecht W, Kluth D: Hereditary multiple atresias of the gastrointestinal tract: report of a case and review of the literature.  J Pediatr Surg. 33(5):794-7, 1998
3- Federici S, Domenichelli V, Antonellini C, Domini R: Multiple intestinal atresia with apple peel syndrome: successful treatment by five end-to-end anastomoses, jejunostomy, and transanastomotic silicone stent. J Pediatr Surg. 38(8):1250-2, 2003
4- Burjonrappa SC, Crete E, Bouchard S: Prognostic factors in jejuno-ileal atresia. Pediatr Surg Int. 25(9):795-8, 2009
5- Guzman MA, Prasad R, Duke DS, de Chadaravian JP: Multiple intestinal atresias associated with angiodysplasia in a newborn. J Pediatr Surg. 46(7):1445-8, 2011
6- Lee SH, Cho YH, Kim HY, Park JH, Byun SY: Clinical experience of complex jejunal atresia. Pediatr Surg Int. 28(11):1079-83, 2012

First Branchial Cleft Anomaly

First branchial cleft anomalies (FBCA) are very rare frequently overlooked and mismanaged. First branchial cleft anomaly is the result of incomplete closure of the cleft formed in the development of the lower face and neck during the 4th to 7th weeks of human development. FBCA has a closed relationship to the parotid and facial nerve. Anatomically two types of FBCA are described: Type 1 with defect in the parotid region, of ectodermal origin arising from duplication of the membranous external auditory canal appearing as sift cysts lined by squamous epithelium. Type 2 more commonly found in children is a defect in the anterior cervical triangle communicating with the external auditory canal, ectodermal and mesodermal in origin, containing skin with adnexal structures as well as cartilage. They present as cyst, sinus, fistula or combinations with opening in the region of the submental triangle. FBCA can present later in life. Recurrent and chronic otorrhea or otitis externa is the most frequent symptom. Other presentations include recurrent periauricular swelling, a sinus in the neck, sinus in external auditory meatus presenting with discharge or fistula below the angle of the mandible.  Some are associated with a myringeal web, an epidermal structure that extends from the floor of the external auditory canal to the umbo of the tympanic membrane. Type 1 cysts can be removed via a retroauricular incision. Type 2 excision needs early identification of the facial nerve at the stylomastoid foramen or proximally in the temporal bone. Many cases present as an infected abscess in the region of Pochet's triangle where they are recurrently incised and drained sometimes not considering the diagnosis of a FBCA. CT Scan can confirm the diagnosis showing the wide tract near the external auditory canal. If there an opening sinus a fistulogram can be performed. Aim of management is complete removal of the lesion with preservation of the facial nerve. Recurrence occurs with infection, incomplete resection and non-curative interventions.   

1- Tham YS, Low WK: First branchial cleft anomalies have relevance in otology and more. Ann Acad Med Singapore. 34(4):335-8, 2005
2- Liu Y, Li T, Xue J, Jia J, Xiao S, Zhao E: First branchial cleft fistula presenting with internal opening on the Eustachian tube: Illustrated cases and literature review. Int J Pediatr Otorhinolaryngol. 76(5):642-5, 2012
3- Do JB, Rasgon BM, Gottschall JA: Congenital pharyngo-oto-cutaneous fistula: surgical management of an unusual anomaly of the first branchial apparatus. Arch Otolaryngol Head Neck Surg. 138(2):189-92, 2012
4- Magdy EA, Ashram YA: First branchial cleft anomalies: presentation, variability and safe surgical
management. Eur Arch Otorhinolaryngol. 270(6):1917-25, 2013
5- Maithani T, Pandey A, Dey D, Bhardwaj A, Singh VP: First branchial cleft anomaly: clinical insight into its relevance in otolaryngology with pediatric considerations.  Indian J Otolaryngol Head Neck Surg. 66(Suppl 1):271-6, 2014
6- Quintanilla-Dieck L, Virgin F, Wootten C, Goudy S, Penn E Jr: Surgical Approaches to First Branchial Cleft Anomaly Excision: A Case Series. Case Rep Otolaryngol. 2016:3902974. doi: 10.1155/2016/3902974. Epub 2016 Feb 29, 2016

PSU Volume 48 No 04 APRIL 2017

Congenital Chylous Ascites

Congenital chylous ascites is a rare and difficult to managed medical condition affecting infants younger than three months characterized by milky ascites with high level of triglycerides. Literature has described three basic causes for the formation of chylous ascites, namely trauma, obstruction of the lymphatic ducts and lymphatic disorders. Lymphatic malformations are the most common cause of chylous ascites in the neonatal period. Obstructive causes include tumors, solid masses, intussusception and malrotation. Diagnosis is obtained by paracentesis and studying the nature of the ascitic fluid characterized by a high level of chylomicrons, triglycerides and lymphocytes. Diagnostic imaging should include US, CT and MRI of the abdomen to exclude conditions needing immediate surgical intervention. Initial management consists of low-fat diet with medium chain triglycerides, since they will be directly absorbed into the portal bloodstream and metabolized into free fatty acids in the liver reducing the lymphatic flow. Should this strategy failed then the child should be placed NPO with total parenteral nutrition along with administering somatostatin analogues for several weeks. Refractory cases to the above-mentioned management should be treated surgically. The main purpose of surgery is identifying a visible point of leakage in the abdominal lymphatic circulation through which lymph leaks into the peritoneal cavity amenable to surgical ligation or occlusion. Lipophilic dyes (Sudan III) or high-fat diets should be given preoperatively to facilitate visualization of the sites of lymphatic leakage. Lymphoscintigraphy is traumatic, difficult to perform in small children, expensive and lacks accuracy in identifying the site of leakage. Other surgical alternatives include deviating the lymphatic leak to the bloodstream using a peritoneo-venous shunt (Leveen). Shunts of this type obstruct and get infected easily. Other authors have used fibrin glue over a hemostatic oxidized cellulose mesh covering an extensive area of the peritoneum suspected of the leak.  

1- te Pas AB, vd Ven K, Stokkel MP, Walther FJ: Intractable congenital chylous ascites. Acta Paediatr. 2004 Oct;93(10):1403-5.
2- Karagol BS, Zenciroglu A, Gokce S, Kundak AA, Ipek MS: Therapeutic management of neonatal chylous ascites: report of a case and review of the literature. Acta Paediatr. 2010 Sep;99(9):1307-10
3- Spagnol L, Conforti A, Valfra L, Morini F, Bagolan P: Preoperative administration of Sudan III and successful treatment of persistent chylous ascites in a neonate.  J Pediatr Surg. 2011 May;46(5):994-7
4- Moreira Dde A, Santos MM, Tannuri AC, Tannuri U: Congenital chylous ascites: a report of a case treated with hemostatic cellulose and fibrin glue. J Pediatr Surg. 2013 Feb;48(2):e17-9.
5- Purkait R, Saha A, Tripathy I, Roy B: Congenital chylous ascites treated successfully with MCT-Based formula and octreotide. J Indian Assoc Pediatr Surg. 2014 Jul;19(3):175-7.
6- Cao Y, Yan W, Lu L, Tao Y, Lu W, Chen Y, Tang Q, Cai W: Parenteral nutrition combined with rice soup can be a safe and effective intervention for congenital chylous ascites. Asia Pac J Clin Nutr. 2016;25(3):631-5

Congenital H-type Rectourethral Fistula

Congenital H-type rectourethral fistula is a very rare anorectal malformation exclusively described in males characterized by a fistulous tract between the rectum and the urethra with an external anal opening in a normal or ectopic position. Most cases are associated with an atretic, hypoplastic or stenotic anterior urethra. These anomalies are more common in children of Asian origin. Diagnosis of this condition can be difficult to make, can pass alone causing disastrous urological consequences to the child. Affected babies have difficult micturition with passage of meconium through urine, urine per rectum or present with recurrent urinary tract infections. Most useful diagnostic test is a voiding cystourethrogram. Usually the fistula communicates internally with the posterior urethra at the verumontanum. But the fistulous tract can be between the membranous urethra and lower anorectal canal, or higher in the prostatic urethra and rectum. The distal opening may lie in the perineum, anal canal or rectum. The tract is lined with squamous epithelium. Embryologically the fistula is explained by persistence of the "cloacal duct" during division of the cloaca. Misalignment of the Tourneux fold and Rathcke's plicae during partition of the cloaca leads to the development of the fistulous tract. Major associated malformations occurs in almost 60% of affected patients with male carrying a higher incidence of severe cardiac, renal vertebral and gastrointestinal anomalies. Management of this condition has been plague by recurrence of the fistulous tract, multiple surgical procedures and later development of fecal incontinence. For low fistulas an anterior perineal approach of closure is suggested. Higher fistulous tracts will need a protective colostomy and use of an anterior or posterior sagittal approach.       

1- Hong AR, Croitoru DP, Nguyen LT, Laberge JM, Homsy Y, Kiruluta GH: Congenital urethral fistula with normal anus: a report of two cases. J Pediatr Surg. 27(10):1278-80, 1992
2- Rintala RJ, Mildh L, Lindahl H: H-type anorectal malformations: incidence and clinical characteristics.  J Pediatr Surg. 31(4):559-62, 1996
3- Lal P, Gupta A, Krisna A, Taneja K: Congenital H-type urethroanal fistula. Pediatri Surg Int. 13: 193-194, 1998
4- Sharma AK, Kothari SK, Menon P, Sharma A: Congenital H-type rectourethral fistula. Pediatr Surg Int. 18(2-3):193-4, 2002
5- Banu T, Hoque M, Laila K, Ul-Huq A, hanif A: Management of male H-type anorectal malformations. Pediatr Surg Int. 25: 857-861, 2009
6- Sharma S, Gupta DK: Diversities of H-type anorectal malformation: a systematic review on a rare
variant of the Krickenbeck classification. Pediatr Surg Int. 33(1):3-13, 2017

TAP Block

Transversus abdominis plane (TAP) block is a recent and promising anesthesia technique used for pain management following abdominal surgery in children and adults. TAP blocks the sensory nerve supply to the anterior abdominal wall by placing ultrasound-guided a local anesthetic in the transversus abdominis plane. The abdominal wall has three muscle layers: external and internal obliques and transversus abdominis. They are innervated by mixed somatic nerves that course between the transversus abdominis and the internal oblique muscles. Blocking the sensory nerve supply to the anterior abdominal wall with long acting local anesthetics provides effective postoperative analgesia in open surgical procedures in children. The same cannot be conferred for laparoscopic procedures as TAP block has been found with very little benefit over local anesthetic port-site infiltration. There is a lack of clinically significant complications when TAP block is performed in children. The most important complications recognized are peritoneal puncture, visceral puncture and intravascular injection of the local anesthetic utilized causing systemic toxicity. TAP block reduces pain and opiate use in children. Use of higher local anesthetic doses for the TAP block in children does not provide benefits on early pain scores but seems to improve analgesic duration and decrease the need for additional analgesics more than twenty-four hours after surgery. TAP block has been effective as part of multimodal analgesia for children undergoing open inguinal hernia repair with significant attenuation in the neuroendocrine stress response induced by surgery. We need further testing and more randomized trials before encouraging the technique as state of the art in children.

1- Sanderman DJ, Bennett M, Dilley AV, Perczuk A, Lim S, Kelly KJ: Ultrasound-guided transversus abdominis plane blocks for laparoscopic appendicectomy in children: a prospective randomized trial. B J Anaesth. 106(6): 882-886, 2011
2-  Hamill JK, Rahiri JL, Liley A, Hill AG: Rectus sheath and transversus abdominis plane blocks in children: a systematic review and meta-analysis of randomized trials. Paediatr Anaesth. 26(4):363-71, 2016
3- Long JB, Birmingham PK, De Oliveira GS Jr, Schaldenbrand KM, Suresh S: Transversus abdominis plane block in children: a multicenter safety analysis of 1994 cases from the PRAN (Pediatric Regional Anesthesia Network) database. Anesth Analg. 119(2):395-9, 2014
4- Suresh S, Taylor LJ, De Oliveira GS Jr.: Dose effect of local anesthetics on analgesic outcomes for the transversus abdominis plane (TAP) block in children: a randomized, double-blinded, clinical
trial. Paediatr Anaesth. 25(5):506-10, 2015
5- Abu Elyazed MM, Mostafa SF, Abdullah MA, Eid GM: The effect of ultrasound-guided transversus abdominis plane (TAP) block on postoperative analgesia and neuroendocrine stress response in pediatric patients undergoing elective open inguinal hernia repair. Paediatr Anaesth. 26(12):1165-1171, 2016
6- Hernandez MA, Vecchione T, Boretsky K: Dermatomal spread following posterior transversus abdominis plane block in pediatric patients: our initial experience. Paediatr Anaesth. Jan 18. doi: 10.1111/pan. 13034, 2017

PSU Volume 48 No 05 MAY 2017

Intrathyroidal Schwannoma

Benign nonepithelial tumors of the thyroid gland are very rare lesions in children and adults. They include lesions such as vascular tumors, smooth muscle tumors and tumors of nerve origin. Primary Schwannoma, also known as neurilemmoma, of the thyroid gland was first reported in 1964 with most cases seen in the adult population. Schwannomas are peripheral nerve tumors originating from neuronal sheath cells (Schwann cells). They mostly occur in the 40 to 60 years old age groups without sex predilection. They are benign tumors that can be found anywhere in the body with half of them originating in the head and neck region. Neurilemmomas of the neck region arise from the cranial nerves with the vagus nerve or its branches being the most frequently affected followed by the cervical sympathetic chain. In the thyroid gland they arise from the sensory nerves or from autonomic innervation of the gland.  Half of all reported cases of intrathyroidal Schwannomas come from Asia. The clinical presentation of a intrathyroidal schwannoma is a typical palpable non-tender thyroid nodule. Thyroid function tests are within normal limits. They have a slow but progressive growth causing compression of vital structures of the neck. Thyroid scintigraphy demonstrates a cold area within the affected lobe. Ultrasound describes a well-delineated, solid or predominantly solid tumor of low echogenicity with variable cystic degeneration. Fine needle aspiration biopsy of the tumor is diagnostic of the histologic nature of the mass. Two growth patterns are seen within the lesion: a predominantly cellular area composed of spindle-shaped Schwann cells with little stromal matrix (Antoni A type tumor), and a less cellular myxoid area with microcyst formation (Antoni type B tumor). On immunohistochemistry Schwannomas are positive for S100 and Vimentin, and negative for Desmin and SMA. Management of intrathyroidal Schwannomas is either enucleation or total thyroid lobectomy. Intrathyroidal Schwannomas are associated with an excellent prognosis once completely removed. 

1- Gustafson LM, Liu JH, Rutter MJ, Stern Y, Cotton RT: Primary neurilemoma of the thyroid gland: a case report. Am J Otolaryngol. 22(1):84-6, 2001
2- Baglaj M, Markowska-Woyciechowska A, Sawicz-Birkowska K, Dorobisz U: Primary neurilemmoma of the thyroid gland in a 12-year-old girl.  J Pediatr Surg. 39(9):1418-20, 2004
3- Graceffa G, Cipolla C, Florena AM, Gentile I, Pompei G, Latteri MA: Primary schwannoma of the thyroid gland involving the isthmus: report of a case. Surg Today. 43(1):106-9, 2013
4- De Simone B, Del Rio P, Sianesi M: Schwannoma mimicking a neoplastic thyroid nodule. Updates Surg. 66(1):85-7, 2014
5- Dhar H, Dabholkar JP, Kandalkar BM, Ghodke R: Primary thyroid schwannoma masquerading as a thyroid nodule. J Surg Case Rep. 23;2014(9), 2014
6- Chen G(1), Liu Z, Su C, Guan Q, Wan F, Dong B, Bao L, Zhang W, Wang Y, Wang G: Primary peripheral nerve sheath tumors of the thyroid gland: A case report and literature review. Mol Clin Oncol. 4(2):209-210, 2016


Glucagonoma is a rare neoplasm of the pancreatic neuroendocrine islet alpha-cells where they secrete abundant glucagon occurring in one of every 20 million individuals. Glucagonoma tumors excessive secretion of proglucagon-derived peptides is clinically characterized by a necrolytic migratory erythema (NME), diabetes mellitus, weight loss, anemia, painful glossitis, stomatitis, thromboembolic complications, dilated cardiomyopathy and neuropsychiatric disturbances. Vast majority of glucagonomas are sporadic and occurs in adults. Children with MEN type 1 can harbor this tumor. Median time between onset of symptoms and diagnosis is 3-4 years. Glucagon hypersecretion increase hepatic glucose output antagonizing the effect of insulin and causing Diabetes. Also it exerts a catabolic role attenuating protein synthesis. Glucagonoma syndrome is the triad of glucagon-secreting tumor, diabetes and NME. The NME is the most specific manifestation of glucagonoma with early recognition leading to a rapid diagnosis of the presence of a glucagon-producing tumor. NME distributed in the groin, perineum and distal extremity is characterized by an annular pattern of erythema and centrally formed fragile vesicles, bullae and crusts present in 70% of patient with glucagonoma. Glucagonoma is a slow growing and low malignancy tumor. Metastasis represent the main prognostic factor for glucagonoma with 100% survival in cases without metastasis.  Metastasis occur to the liver and peripancreatic lymph nodes. Somatostatin analog therapy may be useful in relieving glucagonoma syndrome by inhibiting glucagon secretion and counteracting its effect. CT-Scan is the diagnostic modality to diagnosed a glucagon producing tumor of the pancreas. Glucagonoma typically occurs in the distal pancreas. Fasting glucagon levels are elevated. Complete resection of the primary pancreatic tumor and limiting metastasis, including liver transplantation, is the only chance of cure.   

1- Wei J, Lin S, Wang C, Wu J, Qian Z, Dai C, Jiang K, Miao YI: Glucagonoma syndrome: A case report. Oncol Lett. 10(2):1113-1116, 2015
2- Wewer Albrechtsen NJ, Challis BG, Damjanov I, Holst JJ: Do glucagonomas always produce glucagon? Bosn J Basic Med Sci. 16(1):1-7, 2016
3- Al-Faouri A, Ajarma K, Alghazawi S, Al-Rawabdeh S, Zayadeen A: Glucagonoma and Glucagonoma Syndrome: A Case Report with Review of Recent Advances in Management. Case Rep Surg. Volume 2016;Article ID:1484089
4- Dimitriadis GK, Weickert MO, Randeva HS, Kaltsas G, Grossman A: Medical management of secretory syndromes related to gastroenteropancreatic neuroendocrine tumours. Endocr Relat Cancer. 23(9):R423-36, 2016
5- Han X, Wang D, Kuang T, Rong Y, Lou W: Glucagonoma syndrome: report of one case. Transl Gastroenterol Hepatol. 2016 Sep 19;1:70. doi: 10.21037/tgh.2016.09.01. eCollection 2016.
6- Rodriguez G, Vargas E, Abanza C, Caceres S: Necrolytic migratory erythema and pancreatic glucagonoma. Biomedica. 3;36(2):176-81, 2016


Vipoma is a very rare malignant neuroendocrine tumor. Most Vipomas arise in the pancreas with 10% of them arising from other tissues of neural crest origin of the body. In children a ganglioneuroblastoma can behave as a Vipoma. Most neurogenic tumors associated with the Vipoma syndrome have been found in children. Vipomas secrete vasoactive intestinal peptide (VIP), a hormone which stimulates adenosines 3',5'-cyclic phosphate (cAMP) production by the intestinal tract causing watery diarrhea, hypokalemia, hypophosphatemia, hypomagnesemia, hyperchloremic metabolic acidosis from severe intestinal loss of bicarbonate and achlorydia syndrome due to inhibition of gastric acid production. Occasionally hypercalcemia due to release of PTH by the tumor, glucose intolerance and hypotension can occur. With the diarrhea the patient can develop  flushing similarly to the carcinoid syndrome. Majority of Vipomas are sporadic cases and 50-60% have metastasized by the time the diagnosis is made. VIP is elevated in all cases of Vipomas and can be measured in blood. Diagnosis can be confirmed using imaging such as US, CT-Scan (hyperattenuating lesion in the arterial phase that becomes inconspicuous in the venous phase), MRI, Somatostatin-receptor scintigraphy or PET-Scan. Vipomas appear as well-defined homogenous mass with central necrosis and hypervascularized. Most Vipomas tumors in the pancreas occur in the tail. Surgical extirpation is the mainstay of treatment of Vipomas. If a tumor has been identified, complete surgical excision is the primary form of treatment. If the tumor cannot be removed completely surgical debulking may have a palliative effect. Medical therapy with somatostatin analogue can be used for symptomatic relieve in cases of inability to remove the tumor completely. Others alternatives include peptide receptor radionuclide therapy, streptotozin chemotherapy, ablation, hepatic artery embolization or liver transplant.     

1- Adam N, Lim SS, Ananda V, Chan SP: VIPoma syndrome: challenges in management. Singapore Med J. 51(7):e129-32, 2010
2- Camera L, Severino R, Faggiano A, Masone S, Mansueto G, Maurea S, Fonti R, Salvatore M: Contrast enhanced multi-detector CT and MR findings of a well-differentiated pancreatic vipoma. World J Radiol. 28;6(10):840-5, 2014
3- Vinik A: Vasoactive Intestinal Peptide Tumor (VIPoma). In: De Groot LJ, Chrousos G, Dungan K, Feingold KR, Grossman A, Hershman JM, Koch C, Korbonits M, McLachlan R, New M, Purnell J, Rebar R, Singer F, Vinik A, editors. Endotext [Internet]. South Dartmouth (MA):, Inc.; 2000-.2013 Nov 28.
4- Chen Y, Shi D, Dong F, Han SG, Qian ZH, Yang LI, Wang Y, Yu RS, Li QH, Fu YB: Multiple-phase spiral CT findings of pancreatic vasoactive intestinal peptide-secreting tumor: A case report. Oncol Lett. 10(4):2351-2354, 2015
5- Mark J, Bush S, Glazer E, Strosberg J, Saglam O, Apte SM: Metastatic VIPoma presenting as an ovarian mass. Int J Surg Case Rep. 17:167-9, 2015
6- Zhang X, Zhou L, Liu Y, Li W, Gao H, Wang Y, Yao B, Jiang D, Hu P: Surgical resection of vasoactive intestinal peptideoma with hepatic metastasis aids symptom palliation: A case report. Exp Ther Med. 11(3):783-787, 2016

PSU Volume 48 NO 06 JUNE 2017

Epigastric Artery Flap Extremity Reconstruction

Through and through traumatic defects in the hands and feet of children produced by high-energy penetrating injuries are considered difficult surgical problems requiring complex reconstruction of affected bone, nerves, tendon and associated vascular structures. After a series of debridement procedures surgeons need to provide skeletal fixation and stable coverage. Form, function and safety of each reconstructive option should be considered carefully and weighted against each other when considering a reconstructive plan. Free flap transfer has become the preferred treatment option for reconstruction of the damaged extremities. The rectus abdominis muscle flap and free latissimus dorsi flap with their sizable areas and large vascular pedicles are the most commonly performed. Disadvantage of using this flap is the sacrifice of important muscles that may lead to functional deficit and potential donor site morbidity. The deep inferior epigastric artery perforator (DIEP) flap has been used extensively in breast reconstruction. It provides a huge amount of skin and soft tissue coverage with minimal donor morbidity. The DIEP flap has been utilized in extremity reconstruction, including foot and ankle, thumb reconstruction and repair of massive lower limb soft tissued defects.  Advantages of the DIEP flap include: no need to sacrifice the abdominal musculature, provides a longer pedicle allowing tension free anastomoses and has a reliable and safe vascular supply. The free DIEP flap is suitable for any type of head, neck and extremity defect. The dissection of perforator's vessel is the key to achieve the successful free DIEP flap transfer in children. The US Doppler is still the most effective and economic method to locate the perforators. The overall survival rate of the flap is 96%. The venous outflow is easier to be compromised because of slower flow and thin vessels wall. Disadvantage includes fat hypertrophy and the scar left in the abdominal wall.

1- Hocaolu E, Emekla, Azmeca, Uasar A: Suprafascial pre-expansion of perforator flaps and the effect of pre-expansion on perforator artery diameter. Microsurgery. 34(3):188-96, 2014
2- Kamath BJ, Verghese T, Bhardwaj P: "Wing flaps": perforator-based pedicled paraumbilical flaps for skin defects in hand and forearm. Ann Plast Surg. 59(5):495-500, 20017
3- Stevenson TR, Hester TR, Duus EC, Dingman RO: The superficial inferior epigastric artery flap for coverage of hand and forearm defects. Ann Plast Surg. 12(4):333-9, 1984
4- Stern HS, Nahai F: The versatile superficial inferior epigastric artery free flap. Br J Plast Surg. 45(4):270-4, 1992
5- Choi JY, Chung KC: The Combined Use of a Pedicle Superficial Inferior Epigastric Artery Flap and a Groin Flap for Reconstruction of a Dorsal and Volar Hand Blast Injury. Hand 3:375-380, 2008
6- Tang J, Fang T, Song D, Liang J, Yu F, Wang C: Free deep inferior epigastric artery perforator flap for reconstruction of soft-tissue defects in extremities of children. Microsurgery. 33(8):612-9, 2013

Recurrent Achalasia after Esophagomyotomy

Achalasia is the most common motility disorder of the esophagus causing dysphagia due to loss of primary esophageal peristalsis and impaired relaxation of the lower esophageal sphincter (LES). Balloon dilatation and Botox injection are considered short-lived method of managing achalasia. Effective long-term management of achalasia results with laparoscopic Heller esophagomyotomy and Dor partial fundoplication. Factors known before the procedure such as patient characteristic, degree of esophageal dilatation or tortuosity, manometry findings and prior treatments have little effect on long-term outcome. Common causes of surgical failure are gastroesophageal reflux and recurrent dysphagia. When dysphagia occurs after myotomy is more often recurrent than persistent. Changes in the esophagus and/or LES that develops after the operation is more important in defining recurrence of symptoms. Almost 25% of all patients still experience dysphagia once per week after surgery. It is believed obstructive scar tissue or distortion of the myotomy develop in theses cases. The only predictor of the need for postop dilation is history of preop dilation. Specific causes of recurrent achalasia after surgery include: 1- Incomplete myotomy or scarring of the distal edge of the myotomy. Longer and more separated myotomy reduces this problem; 2- Not performing a fundoplication. This causes abnormal symptoms of reflux and heartburn. A 360-degree fundoplication aggravates dysphagia. Partial fundoplication prevents reflux and does not impair esophageal emptying; 3- GE reflux is considered a common cause of recurrent dysphagia due to esophagitis, scarring and development of Barrett's esophagus; 4- the effects of previous treatment due to scar tissue created from endoscopic manipulation or Botox injections; 5- Esophageal cancer since achalasia patients are at increased risk of developing squamous cell carcinoma. Diagnostic evaluation of recurrent symptoms must include: barium swallow, upper endoscopy and manometry. Management of recurrent achalasia include pneumatic balloon dilatation, revisional surgery, POEM or esophagectomy.  

1- Carter JT, Nguyen D, Roll GR, Ma SW, Way LW: Predictors of long-term outcome after laparoscopic esophagomyotomy and Dor fundoplication for achalasia. Arch Surg. 146(9):1024-8, 2011
2- Franklin AL, Petrosyan M, Kane TD: Childhood achalasia: A comprehensive review of disease, diagnosis and therapeutic management. World J Gastrointest Endosc. 16;6(4):105-11, 2014
3- Patti MG, Allaix ME: Recurrent symptoms after Heller myotomy for achalasia: evaluation and treatment. World J Surg. 39(7):1625-30, 2015
4- Aquino JL, Said MM, Pereira DA, Leandro-Merhi VA, Nascimento PC, Reis VV: Early and Late Assesment of Esophagocardioplasty in the Surgical Treatment of Advanced Recurrent Megaesophagus. Arq Gastroenterol. 53(4):235-239, 2016
5-Saleh CM, Ponds FA, Schijven MP, Smout AJ, Bredenoord AJ: Efficacy of pneumodilation in achalasia after failed Heller myotomy. Neurogastroenterol Motil. 28(11):1741-1746, 2016
6- Fumagalli U, Rosati R, De Pascale S, Porta M, Carlani E, Pestalozza A, Repici A: Repeated Surgical or Endoscopic Myotomy for Recurrent Dysphagia in Patients After Previous Myotomy for Achalasia.  J Gastrointest Surg. 20(3):494-9, 2016

Nontuberculous Mycobacteria Lymphadenitis

Atypical mycobacteria, also known as nontuberculous mycobacteria (NTM) are acid-fast bacteria other than Mycobacterium tuberculosis. Nontuberculous mycobacteria can cause difficult to diagnosed lymphadenitis in immunocompetent children. Exposure of the human oral cavity and respiratory tract to NTM comes from soil, specially after putting wet dirt or soil into their mouths. Incubation periods are variable but can reach five years in some cases. A diagnosis of NTM lymphadenitis should be suspected in children less than five years of age, female predominance, with subacute, unilateral, non-tender cervicofacial lymphadenitis resistant to standard antibiotic therapy. Submandibular and anterior cervical lymph are most commonly involved. Diagnosis is established by acid-fast staining, mycobacterial culture and histopathology. Sampling method for diagnosis includes FNA aspiration, curettage, drainage or complete excision. Polymerase-chain reaction testing of lymph node material has the highest diagnostic yield, followed by mycobacterial culture and microscopy for acid-fast bacilli. Positive culture will confirm the diagnosis but it can take six weeks. Growth characteristic of NTM lymphadenitis include slow-growing (M Fortuitum, Chelonei and Abscessus) and fast-growing mycobacteria (M. Marinum, Kansasii, Avium-intracellulare). In the US the majority of NTM lymphadenitis in children are caused by M Avium complex. A PPD skin test might be positive. The course of NTM lymphadenitis might be variable and involve eruption of the lymph node and tract formation with drainage; the lymph node might also remain indurated. Systemic symptoms are unusual in immunocompetent children. Reactivation of NTM can occur after trauma or injury near the affected area. Management of NTM lymphadenitis includes surgical (complete resection is gold standard if technically feasible), less likely prolonged antimycobacterial oral therapy (Clarithromycin is preferred).  

1- Krantz AM, Varnam M, Fernandez C: Nontuberculous Mycobacteria Lymphadenitis: A Case Report. Cureus. 8(10):e846, 2016
2- Rives P, Joubert M, Launay E, Guillouzouic A, Espitalier F, Malard O: Cervicofacial non-tuberculous mycobacteria: A report of 30 cases. European Ann Otorrhinolaryn Head & Neck disease. 133: 107-111, 2016
3- Garcia-Marcos PW, Plaza-Fornieles M, Menasalvas-Ruiz A, Ruiz-Pruneda R, Paredes-Reyes P, Miguelez SA: Risk factors of non-tuberculous mycobacterial lymphadenitis in children: a case-control study. Eur J Pediatr. doi: 10.1007/s00431-017-2882-3, 2017
4- Al Yazidi LS, Marais BJ, Hazelton B, Outhred A, Kesson A: Nontuberculous Mycobacteria in Children: A Focus on Bloodstream Infections. Pediatr Infect Dis J. 36(4):374-378, 2017
5- Tebruegge M, Pantazidou A, MacGregor D, Gonis G, Leslie D, Sedda L, Ritz N, Connell T, Curtis N: Nontuberculous Mycobacterial Disease in Children - Epidemiology, Diagnosis & Management at a Tertiary Center. PLoS One. 2016 Jan 26;11(1):e0147513. doi: 10.1371/journal.pone.0147513. eCollection 2016.
6- Naselli A, Losurdo G, Avanzini S, Tarantino V, Cristina E, Bondi E, Castagnola E: Management of nontuberculous mycobacterial lymphadenitis in a tertiary care children's hospital: A 20year experience.  J Pediatr Surg. 52(4):593-597, 2017

Journal Club