VOLUME 45, 2015

PSU Volume 45 NO 01 JULY 2015

Transanal Endoscopic Microsurgery

Transanal endoscopic microsurgery (TEM) refers to a minimally invasive technique using an operating proctoscope, a laparoscopic camera and modified laparoscopic instruments to accomplish local removal of low level rectal tumors. The technique is ideal to use to remove anorectal benign adenomas, early stage rectal carcinomas, carcinoids of the rectum, for palliative resection of advanced rectal cancers, and surgically correct rectal prolapse and rectal diverticular disease. Recently TEM has been used to resect presacral tumors in adults. In children TEM has been utilized to resect a presacral rectal duplication cyst. The proctoscope is 4 cm in diameter, has four operating ports, maintains an airtight seal at the anus, with a port for CO2 inflow and outflow facilitating smoke evacuation during cauterization. Due to the restricted space to work the operating instruments are angulated to improve the operator range of motion. Most patients who undergo TEM experience short hospital stay, lower rate of postoperative complications and early return to routine activities even after resection of very large lesions with full thickness excision. Anorectal function short of causing decreased anal sphincter pressures and compliance after surgery seldom causes permanent damage such as fecal incontinence. Compared with preoperative levels there is no significant change in anal squeeze pressure after surgery. Tumors larger than 4 cm caused significant decline in rectal sensitivity, urge to defecate threshold and maximum tolerated volume. TEM excision of adenomas results in low adenoma recurrence rates due to higher negative margin rates. Major complications though low include perforation, bleeding and fistulas. The need for diversion before the procedure has been questioned since the oncologic or infectious morbidity associated with opening the peritoneal cavity is low. The use of TEM in managing more advanced rectal must be tested within formal clinical trials before adopting as routine practice.

1- Heidary B, Phang TP(1), Raval MJ(1), Brown CJ(1): Transanal endoscopic microsurgery: a review. Can J Surg. 57(2):127-38, 2014
2- Guerrieri M, Gesuita R, Ghiselli R, Lezoche G, Budassi A, Baldarelli M: Treatment of rectal cancer by transanal endoscopic microsurgery: experience with  425 patients. World J Gastroenterol. 28;20(28):9556-63, 2014
3- Cunningham C: Transanal endoscopic microsurgery. Recent Results Cancer Res. 203:31-8, 2014
4- Marks JH, Frenkel JL, Greenleaf CE, D'Andrea AP: Transanal endoscopic microsurgery with entrance into the peritoneal cavity: is it safe? Dis Colon Rectum. 57(10):1176-82, 2014
5- Mora Lopez L, Serra Aracil X, Hermoso Bosch J, Rebasa P, Navarro SotoS: Study of anorectal function after transanal endoscopic surgery.  Int J Surg. 13:142-7, 2015
6-Clancy C, Burke JP, Albert MR, O'Connell PR, Winter DC: Transanal endoscopic microsurgery versus standard transanal excision for the removal of rectal neoplasms: a systematic review and meta-analysis. Dis Colon Rectum. 58(2):254-61, 2015

Fitz-Hugh-Curtis Syndrome

Fitz-Hugh-Curtis syndrome (FHCS) is a rare complication of pelvic inflammatory disease. It results from ascending pelvic infection causing liver capsule inflammation and right upper quadrant pain mostly in reproductive-aged female with sexual activity characterized by violin-string like perihepatic adhesions between the liver capsule and diaphragm or anterior peritoneal surface. The incidence ranges from 4% to 27% in women with pelvic inflammatory disease. FHCS can be seen in 4% of  adolescent females with pelvic inflammatory disease. Predominant symptoms are pain in the right upper quadrant, tenderness, fever and pleuritic right-sided pain. Symptoms can be confused with biliary disease. On physical exam patients can be positive for cervical motion tenderness and adnexal tenderness. Neisseria gonorrhoeae and Chlamydia trachomatis are the primary causative agents with this last one as a more common causative organism. In women, the inflammation of the liver capsule has been attributed to the direct bacterial spread from an infected fallopian tube via the right paracolic gutter. In men, hematogenous and lymphatic spread to liver has been postulated as the underlying mechanism of spread. CT scan may show subcapsular fluid collection, thickening of hepatic capsule in the arterial phase, and wedging enhancement of the involved liver parenchyma in more than 50% of patients. Definitive diagnosis needs invasive procedure like laparoscopy or laparotomy. Most cases of FHCS are managed with antibiotics against the principal offending bacteria. If symptoms persist then surgical lysis of adhesions should be considered. Laparoscopy has both diagnostic and therapeutic benefits.

1-  Banikarim C, Chacko MR: Pelvic inflammatory disease in adolescents. Adolesc Med Clin. 15(2):273-85, 2004
2- Hyun JJ, Kim JY, Bak YT, Lee CH, Choi SY: Education and imaging. Gastrointestinal: Fitz-Hugh-Curtis syndrome. J Gastroenterol Hepatol. 21(9):1493, 2006
3- Risser WL, Risser JM, Benjamins LJ, Feldmann JM: Incidence of Fitz-Hugh-Curtis syndrome in adolescents who have pelvic inflammatory disease. J Pediatr Adolesc Gynecol. 20(3):179-80, 2007
4- Hong DG, Choi MH, Chong GO, Yi JH, Seong WJ, Lee YS, Park IS, Cho YL: Fitz-Hugh-Curtis Syndrome: single centre experiences.  J Obstet Gynaecol. 30(3):277-80, 2010
5- Perricone G: Hepatology: Fitz-Hugh-Curtis syndrome: pelvic inflammatory disease with perihepatitis. J Gastroenterol Hepatol. 29(11):1853, 2014
6- You JS, Kim MJ, Chung HS, Chung YE, Park I, Chung SP, Kim S, Lee HS: Clinical features of Fitz-Hugh-Curtis Syndrome in the emergency department. Yonsei Med J. 53(4):753-8, 2012

Spontaneous Pneumomediastinum

Pneumomediastinum can have diverse etiology in the pediatric population such as chronic lung disease (asthma), rapid scuba diver ascent, illicit drug use, foreign body ingestion and blast injury. There is a significant group of children with spontaneous pneumomediastinum (SPM) and no apparent etiology. The SPM develops after alveolar rupture following an acute rise in intrathoracic pressure. Air escapes from the alveoli to the interstitium and then tracks along the tracheobronchial tree after a pressure gradient between the lung periphery and mediastinum. Clinically the child develops chest pain radiating to neck or back, shortness of breath, hoarseness,  coughing or subcutaneous emphysema. Asthmatics have a higher incidence of spontaneous pneumomediastinum caused by inspiratory effort against a blocked airway (Mueller Maneuver). Weight lifting and sport related are some other instigating factor. Other times inhalation of illicit drugs is a predisposing factor. In such situation the workup imposed in patients with SPM has included CT-Scan, esophagogram, bronchoscopy, serial chest films, etc., when most cases resolve spontaneously without significant intervention. Most of this cases can be initially managed with a short period of observation in the emergency department followed by rest under the care at home of a responsible guardian. Invasive studies or esophagography should be performed on an individual and not systematic basis such as history of emesis, dysphagia/odynophagia, radiographic findings of pleural effusion or atelectasis, etc. The hospital course is usually benign in cases of SPM and most patients require only supportive measures. The risk of recurrence is very low except in cases with chronic lung disease.

1- Lee CY, Wu CC, Lin CY: Etiologies of spontaneous pneumomediastinum in children of different ages. Pediatr Neonatol. 50(5):190-5, 2009
2- Chen IC, Tseng CM, Hsu JH, Wu JR, Dai ZK: Spontaneous pneumomediastinum in adolescents and children. Kaohsiung J Med Sci. 26(2):84-8, 2010
3- Lee CY, Wu CC, Lin CY: Etiologies of spontaneous pneumomediastinum in children in middle Taiwan. Pediatr Pulmonol. 45(9):869-73, 2010
4- Wong KS, Wu HM, Lai SH, Chiu CY: Spontaneous pneumomediastinum: analysis of 87 pediatric patients. Pediatr Emerg Care. 29(9):988-91, 2013
5- Bakhos CT, Pupovac SS, Ata A, Fantauzzi JP, Fabian T: Spontaneous pneumomediastinum: an extensive workup is not required. J Am Coll Surg. 219(4):713-7, 2014
6- Fitzwater JW, Silva NN, Knight CG, Malvezzi L, Ramos-Irrizary C, Burnweit CA: Management of spontaneous pneumomediastinum in children. J Pediatr Surg. 50(6): 983-986, 2015

PSU Volume 45 NO 02 AUGUST 2015


Anterior Cutaneous Nerve Entrapment Syndrome (ACNES) is a recent identified cause of chronic abdominal pain in children and adults. The superficial end branches of intercostal thoracic nerves twigs are somehow trapped at the level of the rectus abdominal muscle leading to pain, and in a few occasions  vomiting. The exact pathophysiology of ACNES is unknown, but is probably related to traction or compression of the anterior portions of intercostal thoracic nerves, usually involving intercostal thoracic nerve VIII to XII. Pregnancy, abdominal trauma, and chronic nerve root compression caused by thoracolumbar or orthopedic conditions are occasionally identified as predisposing factors. Diagnosis of ACNES is suspected by a constant nagging localized abdominal pain that increases during play or sport associated with a positive Carnett sign. Carnett test is performed as follows: the physician localizes the point of maximal pain with his index finger, and the child is then asked to lift the head or upper torso or the legs while the palpating index finger remains on the painful spot. If this lifting aggravates the pain, its origin is probably located in the abdominal wall. Hypoesthesia, hyperesthesia/ algesia or sometimes even allodynia may be found by comparison to the normal contralateral abdominal side. A positive pinch test is a sensitive and highly underrated sign reflecting the presence of a neuropathic pain syndrome such as ACNES. Attenuating the pain using trigger point local anesthetic infiltration support the diagnosis. These children undergo extensive laboratory and imaging workup which are usually negative and expensive.  Management consists of pain medication. If pain persists, US-guided transverse abdominis plane technique blocking the entrapped nerve using subfascial lidocaine with steroid infiltration is utilized. With recurrent or recalcitrant pain an anterior cutaneous neurectomy will be needed. The neurectomy removes the affected nerve end twigs. This type of surgical procedure is highly successful in children and adults.

1-  Scheltinga MR, Boelens OB, Tjon A Ten WE, Roumen RM: Surgery for refractory anterior cutaneous nerve entrapment syndrome (ACNES) in children. J Pediatr Surg. 46(4):699-703, 2011
2- Boelens OB, Scheltinga MR, Houterman S, Roumen RM: Management of anterior cutaneous nerve entrapment syndrome in a cohort of 139 patients. Ann Surg. 254(6):1054-8, 2011
3- Boelens OB, van Assen T, Houterman S, Scheltinga MR, Roumen RM: A double-blind, randomized, controlled trial on surgery for chronic abdominal pain due to anterior cutaneous nerve entrapment syndrome. Ann Surg. 257(5):845-9, 2013
4- Akhnikh S, de Korte N, de Winter P: Anterior cutaneous nerve entrapment syndrome (ACNES): the forgotten diagnosis. Eur J Pediatr. 173(4):445-9, 2014
5- Nizamuddin SL, Koury KM, Lau ME, Watt LD, Gulur P: Use of targeted transversus abdominus plane blocks in pediatric patients with anterior cutaneous nerve entrapment syndrome. Pain Physician. 17(5):E623-7, 2014
6- Bairdain S, Dinakar P, Mooney DP: Anterior Cutaneous Nerve Entrapment Syndrome in Children. J Pediatr Surg 50(7): 1177-1179, 2015


Achalasia is a rare motility disorder of the esophagus due to absence of peristalsis in the esophageal body impairing relaxation of the lower esophageal sphincter during swallowing. The etiology of achalasia is  likely to be affected by various factors causing immune-mediated ganglionitis which results in degeneration of the myenteric nerve plexus of the esophageal wall. Achalasia causes regurgitation, dysphagia to solids, retrosternal pain and weight loss. The diagnosis of achalasia is first suggested in a barium swallow and corroborated using esophageal manometry studies. Achalasia is either managed by esophageal balloon dilatation and most effectively with either open or laparoscopic anterior surgical esophageal myotomy. Peroral endoscopic myotomy (POEM) is a novel procedure developed recently which has ameliorated the symptoms of patients with achalasia. The endoscopic procedure, performed in the operating room under general anesthesia, consists of entry incision on the mucosa at the level of the gastroesophageal junction, establishing a tunnel on the submucosa, performing the myotomy followed by sealing of the entry incision. The myotomy is primary performed for the circular muscle fibers or both the circular and longitudinal muscle fibers. Leak through the mucosal seal can cause peritonitis and/or mediastinitis. Compared with the surgical approach to achalasia, POEM has the advantage of minimally invasiveness. POEM can significantly improve esophageal motility by decreasing upper esophageal sphincter and lowering esophageal sphincter pressure. POEM has no effect on esophageal body peristalsis. After POEM incomplete myotomy and gastroesophageal reflux can become a problem. The role of POEM in the treatment of other esophageal motor disorders such as diffuse esophageal spasm, non-relaxing hypertensive LES and nutcracker esophagus is still debatable. POEM is a safe and effective technique for treating pediatric achalasia.

1- Allaix ME, Patti MG: Endoscopic Dilatation, Heller Myotomy, and Peroral Endoscopic Myotomy: Treatment  Modalities for Achalasia. Surg Clin North Am. 95(3):567-578, 2015
2- Zhang Y, Ling-hu E, Zhai Y, Peng L, Wang X: Peroral endoscopic myotomy plus balloon shaping for achalasia: a preliminary study. Hepatogastroenterology. 62(137):82-6, 2015
3- Tang X, Gong W, Deng Z, Zhou J, Ren Y, Zhang Q, Chen Z, Jiang B: Usefulness of peroral endoscopic myotomy for treating achalasia in children: experience from a single center. Pediatr Surg Int. 31(7):633-8, 2015
4- Kumbhari V, Khashab MA: Peroral endoscopic myotomy. World J Gastrointest Endosc. 7(5):496-509, 2015
5- Yao S, Linghu E: Peroral endoscopic myotomy can improve esophageal motility in patients with
achalasia from a large sample self-control research (66 patients). PLoS One. 10(5):e0125942, 2015
6- Ramchandani M, Reddy DN, Darisetty S, Kotla R, Chavan R, Kalpala R, Galasso D, Lakhtakia S, Rao GV: Peroral endoscopic myotomy for Achalasia Cardia: A single center experience of over 200 consecutive patients: treatment analysis and follow up. Dig Endosc. May 27, 2015


Lymphorrhea is an abnormal flow of lymph draining externally from disrupted lymphatics vessels or previously retained within a wound. In children lymphorrhea usually occurs following a surgical procedure for a lymphatic anomaly, following cardiothoracic surgery, in penetrating trauma to the neck or during  removal of lymph nodes for diagnostic or therapeutic purposes. Complications of uncontrolled lymphorrhea can lead to subcutaneous collection of lymphatic fluid or a draining fistula. High output lymphatic leakage is associated with increased mortality when intervention is not initiated in a timely fashion due to critical loss of fluids, proteins and electrolytes causing lymphocyte and antibody depletion Most cases of lymphorrhea in children occur after surgery for lymphangioma or cystic hygroma, a benign condition of the lymphatic system consisting of cysts of varying size. Initial management of lymphorrhea consists of diet modification, drainage, pressure dressings, and reoperation to stop lymphatic drainage. Octeotride, a somatostatin analog has been used successfully to manage port-operative lymphorrhea in children after removal of large lymphangioma in the cervical and axillary region. Midodrine, an oral selective alpha-adrenergic drug, can affect contraction of smooth muscle and decrease lymphatic flow. Sclerotherapy using either doxycycline, ethanol, bleomycin or minocycline has also been successfully used to manage lymphatic leakage. Surgical exploration to occlude lymphatic vessels with the use of fibrin sealant has also produce  benefit in selected patients. 

1- Suver DW, Perkins JA, Manning SC: Somatostatin treatment of massive lymphorrhea following excision of a lymphatic malformation. International J Pediatr Otorhinolaryngology. 68: 845-850, 2004
2- Carcoforo P, Soliani G, Maestroni U: Octeotride in the Treatment of Lymphorrhea after Axillary Node dissection. A prospective randomize controlled trial.J Am Coll Surg. 196:365–369, 2003
3- Lemaitre J, Segers B, Lebrun E: [The use of lanreotide in the management of lymphorrhea after an aortic valve replacement]. Interactive Cardiovascular and Thoracic Surgery 15:762-763, 2012
4- Friedmann MD: Evaluation and Management of Lymhadenopathy in Children. Pediatrics in Review. 29(2): 53-60, 2008
5- Miloundja J, Manfoumbi Ngoma AB, Mba Ella R, Nguema Edzang B, N'Zouba L: [Cystic cervicofacial lymphangioma in children in Gabon]. Ann Otolaryngol Chir Cervicofac. 124(6):277-84, 2007
6- Cuervo JL, Galli E, Eisele G, Johannes E, Fainboim A, Tonini S, Joaquin W, Viola B, Nazar M:  [Lymphatic malformations: percutaneus treatment with bleomycin]. Arch Argent Pediatr. 109(5):417-22, 2011

PSU Volume 45 No 03 SEPTEMBER 2015

Subcutaneous Zygomycosis

Subcutaneous zygomycosis, also known as Entomophthoromycosis, is a rare, sporadic subcutaneous fungal infection largely restricted to tropical areas of Africa, Asia and South America. Two clinically distinct form can be found: subcutaneous zygomycosis, caused by Basidiobolus ranarum and rhinofacial zygomycosis caused by Conidiobolus coronatus. Neither of these two forms occurs preferentially in patients with underlying disease nor defective immunity. These fungi are used as bio-insecticides. Subcutaneous zygomycosis is characterized by the formation of firm and non-tender disciform nodule generally appearing on the extremity and trunk mostly in children. The nodule can enlarge spreading locally. Skin is usually tethered to the mass and it may be pigmented. Males are more much frequently affected than females. The mode of infection is by traumatic implantation through abrasion or pricks. The infection can also be transmitted by insect bites or by transepidermal inoculation with contaminated vegetable matter. Subcutaneous zygomycosis can mimic a soft tissue tumor such as synovial sarcoma or Burkitt's lymphoma. Histologically an eosinophilic granulation infiltration is found with broad thin walled infrequently septate hyphal fragments enveloped by eosinophilic "Splendore-Hoeppli" material. Diagnosis of subcutaneous zygomycosis is accepted if the fungus is demonstrated in either histology or culture. Role of surgery in subcutaneous zygomycosis is limited to diagnostic biopsy. Excision leads to recurrence. The standard treatment of choice for subcutaneous zygomycosis is oral potassium iodide or imidazoles in particular itraconazole, ketaconazole and posaconazole.

1- Sivaraman, Thappa DM, Karthikeyan, Hemanthkumar: Subcutaneous phycomycosis mimicking synovial sarcoma. Int J Dermatol. 38(12):920-3, 1999
2- Mahamaytakit N, Singalavanija S, Limpongsanurak W: Subcutaneous zygomycosis in children: 2 case reports. J Med Assoc Thai. 97: Suppl 6:S248-53, 2014
3- Gugnani HC: A review of zygomycosis due to Basidiobolus ranarum. Eur J Epidemiol. 15(10):923-9, 1999
4- Anand M, Deshmukh SD, Pande DP, Naik S, Ghadage DP: Subcutaneous Zygomycosis Due to Basidiobolus ranarum: A Case Report from Maharastra, India. J Trop Med. 2010:950390, 2010
5- Thotan SP, Kumar V, Gupta A, Mallya A, Rao S: Subcutaneous phycomycosis--fungal infection mimicking a soft tissue tumor: a case report and review of literature.  J Trop Pediatr. 56(1):65-6, 2010
6- Raveenthiran V, Mangayarkarasi V, Kousalya M, Viswanathan P, Dhanalakshmi M, Anandi V: Subcutaneous entomophthoromycosis mimicking soft-tissue sarcoma in children. J Pediatr Surg. 50(7):1150-5, 2015

Hypertrophic Scar

Hypertrophic scar (HTS) is a benign hyperproliferative growth of dermal fibroblasts. They occur most commonly after thermal injury to the skin caused by burns and trauma, but can also be seen after elective surgical procedures. Almost 15% of all wounds can develop into a hypertrophic scar. Children with hypertrophic scars experience marked physical (deformity, restricted range of motion, pain and pruritus) and psychological (cosmetic concern) problems. Fibroblasts from hypertrophic scars produce increase amounts of collagen when compared with normal fibroblasts. The undesirable physical properties of HTS tissue can be attributed to the presence of a large amount of extracellular matrix that is of altered composition and organization, compared to normal dermis or mature scar. This matrix is the product of a dense population of fibroblasts, maintained in a hyperactive state by inflammatory cytokines such as TGF-b and other factors, some of which may be physical in origin. Most HTS are accepted by the patient so long as they are asymptomatic. Management of HTS can range from conservative to excision to intralesional injection therapy or a combination of these. The most commonly used therapeutic method for HTS and keloids consist of the steroid triamcinolone acetonide (Kenalog) wound injection. The dosage varies from 10-40 mg/ml with a treatment interval administered every 4-6 weeks until the scar flattens. Kenalog can suppress vascular endothelial growth factor, inhibit fibroblast proliferation, inhibit transforming growth factor and induce scar regression. Varying the dosage can provide good results with less recurrence and complications. Results can be improved and scar recurrence reduced when triamcinolone is combined with other therapies such as 5-Fluorouracil (best results in terms of rapid response and fewer side-effects), surgery, pulsed-dye laser, and radiation therapy.

1- Williams FN, Herndon DN, Branski LK: Where we stand with human hypertrophic and keloid scar models. Exp Dermatol. 23(11):811-2, 2014
2- Wang J, Dodd C, Shankowsky HA, Scott PG, Tredget EE; Wound Healing Research Group: Deep dermal fibroblasts contribute to hypertrophic scarring. Lab Invest. 88(12):1278-90, 2008
3- Darougheh A(1), Asilian A, Shariati F: Intralesional triamcinolone alone or in combination with 5-fluorouracil for the treatment of keloid and hypertrophic scars. Clin Exp Dermatol. 34(2):219-23, 2009
4- Longaker MT: Regenerative medicine: a surgeon's perspective. J Pediatr Surg. 45(1):11-7, 2010
5- Hayashi T, Furukawa H, Oyama A, Funayama E, Saito A, Murao N, Yamamoto Y: A new uniform protocol of combined corticosteroid injections and ointment application reduces recurrence rates after surgical keloid/hypertrophic scar excision. Dermatol Surg. 38(6):893-7, 2012
6- Perdanasari AT, Lazzeri D, Su W, et al: Recent Development in the use of Intralesional Injections Keloid Treatment. Archives Plast Surg 41(6): 620-629, 2014

Annular Pancreas: Late Presentation

Annular pancreas (AP) is a congenital anomaly occurring when a ring of pancreatic tissue encircles the duodenum partially or completely. Infants born with this anomaly can develop duodenal obstruction, stenosis, peptic ulcer, obstructive jaundice, pancreatitis or pancreatic malignancy. Others remain asymptomatic until later in life. Pathogenesis includes hypertrophy of both the ventral and dorsal anlage, adhesion of a portion of the ventral anlage to the duodenum before migration, or fusion of aberrant pancreatic tissue from the duodenum. The annulus is usually comprised of a band of pancreatic tissue, which encircles the second portion of the duodenum. The annulus itself can be complete, partial, intramural, or extramural. AP can be classified according to where the annular duct communicates with Wirsung's (Type 1; most common), common bile duct, papilla or Santorini. Around 50% of all annular pancreas are diagnosed in infancy due to complete duodenal obstruction. Later in life the older child or adult develop chronic partial duodenal obstruction associated with abdominal pain, nausea and postprandial vomiting. Diagnosis relies on images. UGIS might depict an incomplete duodenal obstruction. CT-Scan shows that pancreatic tissue encircles the second portion of the duodenum. ERCP can show the classic features of a pancreatic duct system encircling the duodenum. MRCP is the best non invasive study showing the aberrant pancreatic duct circling and extending to the right of the duodenum. Intraoperative diagnosis remains the best diagnostic modality for annular pancreas in any age group. Annular pancreas with duodenal obstruction should be managed with a duodenal physiological bypass procedure such as duodenoduodenostomy or duodenojejunostomy depending on the individual case. Gastrojejunostomy is an alternative option in case of grossly fibrotic duodenal C-loop, but can lead to marginal ulcers. Local resection of the annular segment is avoided because of the fear of development of pancreatic fistula and pancreatitis.

1- Choi J, Kim MJ, Kim JH, Lim JS, Oh YT, Chung JJ, Song SY, Chung JB, Yoo HS, Lee JT, Kim KW: Annular pancreas: emphasis on magnetic resonance cholangiopancreatography findings. J Comput Assist Tomogr. 28(4):528-32, 2004
2- Fu PF, Yu JR, Liu XS, Shen QY, Zheng SS: Symptomatic adult annular pancreas: report of two cases and a review of the literature. Hepatobiliary Pancreat Dis Int. 4(3):468-71, 2005
3- Zyromski NJ, Sandoval JA, Pitt HA, Ladd AP, Fogel EL, Mattar WE, Sandrasegaran K, Amrhein DW, Rescorla FJ, Howard TJ, Lillemoe KD, Grosfeld JL: Annular pancreas: dramatic differences between children and adults. J Am Coll Surg. 206(5):1019-25, 2008
4- Zilberstein B, Sorbello MP, Orso IR, Cecconello I: Laparoscopic duodenal-jejunal bypass for the treatment of duodenal obstruction caused by annular pancreas: description of a surgical technique. Surg Laparosc Endosc Percutan Tech. 21(2):e60-4, 2011
5- Badiola I, Las Navas E, Sanz A, Estraviz B, Ibanez FJ: Laparoscopic side-to-side duodenoduodenostomy for adult annular pancreas. Cir Esp. 92(9):630-1, 2014
6- Alahmadi R, Almuhammadi S: Annular pancreas: a cause of gastric outlet obstruction in a 20-year-old patient. Am J Case Rep.15:437-40, 2014

PSU Volume 45 NO 04 OCTOBER 2015

Duodenal Web

Intrinsic congenital obstruction of the duodenum is either caused by an atresia, stenosis or web with a prevalence of 1:6000 live births. Congenital duodenal obstruction is the result of several embryologic defects in foregut development, canalization or rotation. Duodenal atresia causes a complete obstruction producing symptoms after birth with evident radiographic findings, while true stenosis and webs of the duodenum can have delayed presentation manifesting later in life. The radiographic signs of duodenal atresia are the "double bubble" with gaseous distension of the stomach and proximal duodenum and total absence of intestinal gas distally. Duodenal webs are the most rare and difficult to diagnose of the intrinsic obstruction occurring in this portion of the gastrointestinal tract. They manifest with recurring and progressive bouts of vomiting, nausea, epigastric discomfort and early satiety. Plain film shows a double bubble effect with distal air. The differential diagnosis includes duodenal stenosis, malrotation with midgut volvulus, pyloric stenosis and gastroesophageal reflux. The diagnosis of a duodenal web can be made with fiberoptic upper endoscopy, hypotonic duodenography or an upper gastrointestinal contrast imaging study. Initial management of any form of intestinal obstruction must include gastric decompression, fluid resuscitation and correction of electrolyte abnormalities, most commonly hypochloremic metabolic alkalosis. There are several varieties of webs: complete duodenal atresias or imperforate webs, intraluminal imperforate webs (wind sock webs), and perforated webs with either central or eccentric apertures. Most duodenal webs are preampullary with a central single aperture and size ranging from 0.5 to 20 mm. Annular pancreas is the most common associated condition of a duodenal web. Surgical management of a duodenal web includes transduodenal web excision with transverse duodenoplasty or bypass duodenoduodenostomy. Complications of the procedure include delayed gastric emptying, pancreatitis, wound infection and duodenal stenosis or leak.

1- Rothenberg SS: Laparoscopic duodenoduodenostomy for duodenal obstruction in infants and
children.  J Pediatr Surg. 37(7):1088-9, 2002
2- Melek M, Edirne YE: Two cases of duodenal obstruction due to a congenital web. World J Gastroenterol. 14(8):1305-7, 2008
3- Mahomed A, D'hondt B, Khan K, Butt A: Technical aspects of the laparoscopic management of a late presenting duodenal web. J Laparoendosc Adv Surg Tech A. Suppl 1:S175-7, 2009
4-Kshirsagar AY, Sulhyan SR, Vasisth G, Nikam YP: Duodenal stenosis in a child. Afr J Paediatr Surg. 8(1):92-4, 2011
5- Sarin YK, Sharma A, Sinha S, Deshpande VP: Duodenal webs: an experience with 18 patients. J Neonatal Surg. 1(2):20, 2012
6- DiMaio CJ, Kamal N, Hogan CM, Midulla PS: Pediatric therapeutic endoscopy: endoscopic management of a congenital duodenal web. Gastrointest Endosc. 80(1):166-7, 2014

Familial Hirschsprung's Disease

A small proportion, probably in the range between 2% and 9% of children born with Hirschsprung's disease (HD), have an inherited familial predisposition, as well as occurring in mono- and dizygotic twins, and a 12% association with chromosomal anomalies. This subgroup of children with familial HD is usually male with an apparent incomplete gene penetrance and variable phenotype. Affected families with HD have  200 times higher risk of recurrence. The length of the aganglionic segment is one of the most consistent predictors of HD transmissibility. Long segment and total colonic aganglionosis is significantly more frequent in familial HD cases than in the sporadic variety carrying the highest risk of recurrence. There is an increase female tendency to transmit the condition to the offspring. The highest recurrence rate occurs in a male sibling of a female proband with long segment HD. RET gene is the major gene causing Hirschsprung's disease (HD). RET proto-oncogene and EDNRB gene variation are identified in 70% of familial HD compared with 30% in the sporadic form. No specific sites on the gene are consistently identified with variations including mutations, frame shifts, deletions, and single nucleotide polymorphism in the RET gene. The combined cumulative effects of the susceptibility loci of RET and EDNRB genes, probably contribute to long-segment and total colonic aganglionosis in familial cases. RET intronic variations may influence gene penetration. Genetic counseling should be offered in these families and in particular for those patients with long segment and total colonic aganglionosis.

1- Schiller M, Levy P, Shawa RA, Abu-Dalu K, Gorenstein A, Katz S: Familial Hirschsprung's disease--a report of 22 affected siblings in four families. J Pediatr Surg. 25(3):322-5, 1990
2- Russell MB, Russell CA, Fenger K, Niebuhr E: Familial occurrence of Hirschsprung's disease. Clin Genet. 45(5):231-5, 1994
3- Moore SW, Zaahl MG: A review of genetic mutation in familial Hirschsprung's disease in South Africa: towards genetic counseling. J Pediatr Surg. 43(2):325-9, 2008
4-Mc Laughlin D, Puri P: Familial hirschsprung's disease: a systematic review. Pediatr Surg Int. 31(8):695-700, 2015
5- Tomuschat C, Puri P: RET gene is a major risk factor for Hirschsprung's disease: a meta-analysis.
Pediatr Surg Int. 31(8):701-10, 2015
6- Moore SW, Zaahl M: Clinical and genetic correlations of familial Hirschsprung's disease. J Pediatr Surg. 50(2):285-8, 2015

Sengstaken-Blakemore Tube

In the 1946, Blakemore and Sengstaken developed a tube with two balloons to control bleeding from esophageal or gastric varices. The tube allows independent inflation of the gastric and esophageal balloon with a distal lumen to aspirate the stomach. With the routine use of modern endoscopic technique the Sengstaken-Blakemore tube (SBT) is rarely use today. The tube is passed down into the esophagus and the gastric balloon is inflated inside the stomach. Traction is applied to the tube so that the gastric balloon will compress the gastroesophageal junction and reduce the blood flow to esophageal or gastric varices. If the use of traction alone cannot stop the bleeding, the esophageal balloon is also inflated to help stop the bleeding. The esophageal balloon should not remain inflated for more than six hours to avoid necrosis. Cooling the SBT has been held to stiffen it and aid during insertion. In a few children bleeding is so severe that medical (octreotide, vasopressin) and endoscopic (sclerotherapy and/or banding) interventions are not feasible, safe or effective and the SBT is required to control blood loss and allow the patient to be resuscitated and stabilized. The SBT can be used effectively in children with bleeding esophagogastric varices from portal hypertension and in the rare case of an aortoesophageal fistula. Hemodynamically unstable patients requiring intubation ventilator support and large volumes of fluids and blood products for catastrophic upper GI bleeding considered unsuitable for first line management should undergo SBT insertion. Almost half of those patients are infants needing a 14 or 16 Fr SBT. In cases of bleeding aortoesophageal fistula the SBT can be lifesaving providing suitable time for surgical repair. Complications of SBT insertion include: recurrent bleeding, tube dislodgement, esophageal rupture, airway compromise, external cardiac compression, aspiration pneumonitis, gastric mucosal ulceration and pressure necrosis at lips and cheeks. Pressure necrosis occurs after more than four days of constant use of the SBT.

1- Jennuvat S, Vithayasai N: Hepatocellular carcinoma in children presents with massive upper gastrointestinal bleeding: a case report. J Med Assoc Thai. 94 Suppl 3:S222-5, 2011
2- Pinto-Marques P, Romaozinho JM, Ferreira M, Amaro P, Freitas D: Esophageal perforation--associated risk with balloon tamponade after endoscopic therapy. Myth or reality? Hepatogastroenterology. 53(70):536-9, 2006
3- Coates LJ, McNally J, Caputo M, Cusick E: Survival in a 2-year-old boy with hemorrhage secondary to an aortoesophageal fistula. J Pediatr Surg. 46(12):2394-6, 2011
4- Singhi S, Jain P, Jayashree M, Lal S: Approach to a child with upper gastrointestinal bleeding. Indian J Pediatr. 80(4):326-33, 2013
5- Feng AC, Liao CY, Fan HL, Chen TW, Hsieh C: The modified Sugiura procedure as bridge surgery for liver transplantation: a case report. J Med Case Rep. 9:50, 2015
6- Jayakumar S, Odulaja A, Patel S, Davenport M, Ade-Ajayi N: Surviving Sengstaken. J Pediatr Surg. 50(7):1142-6, 2015

PSU Volume 45 NO 05 NOVEMBER 2015

Macrophage Activation Syndrome

Macrophage activation syndrome (MAS) is a severe, potentially fatal condition caused by excessive activation and development of macrophages and T-cells (mainly CD8+) leading to an overwhelming inflammatory reaction in the host (cytokine storm). The main clinical manifestation of macrophage activation syndrome includes fever, hepatosplenomegaly, lymphadenopathy, severe cytopenia, liver dysfunction and coagulopathy consistent with disseminated intravascular coagulation. MAS is also associated with extreme hyperferritinemia. A pathognomonic feature of MAS is the expansion of well-differentiated macrophages exhibiting hemophagocytic activity typically found in bone marrow and lymph nodes.  MAS is most strongly associated with the systemic form of juvenile idiopathic arthritis, but it can also occur in patients with systemic lupus erythematosus, Kawasaki disease juvenile dermatomyositis, antiphospholipid syndrome and mixed connective tissue disease. Diagnosis of MAS is difficult due to its resemblance to sepsis. In a child with persistently active underlying rheumatologic disease, a fall in the ESR and platelet count, particularly in a combination with persistently high CRP and increasing levels of serum D-dimer and ferritin, should raise a suspicion of impending MAS. Diagnosis of MAS is usually confirmed by the demonstration of hemophagocytosis in the bone marrow. Treatment options include a pro-apoptotic chemotherapy. Patients with evidence of continued or progressive central nervous system involvement after two weeks of systemic therapy require intrathecal therapy with methotrexate combined with corticosteroid induction followed by hematopoietic stem cell transplantation. The role of the surgeon in this rare condition includes central venous catheter placement and lymph node biopsy as warranted for the treatment and diagnosis of MAS.

1- Janka GE: Hemophagocytic syndromes. Blood Rev. 21(5):245-53, 2007
2- Grom AA, Mellins ED: Macrophage activation syndrome: advances towards understanding pathogenesis. Curr Opin Rheumatol. 22(5):561-6, 2010
3- Lin CI, Yu HH, Lee JH, Wang LC, Lin YT, Yang YH, Chiang BL: Clinical analysis of macrophage activation syndrome in pediatric patients with autoimmune diseases. Clin Rheumatol. 31(8):1223-30, 2012
4- Schulert GS, Grom AA: Macrophage activation syndrome and cytokine-directed therapies. Best Pract Res Clin Rheumatol. 28(2):277-92, 2014
5- Lehmberg K, Pink I, Eulenburg C, Beutel K, Maul-Pavicic A, Janka G: Differentiating macrophage activation syndrome in systemic juvenile idiopathic arthritis from other forms of hemophagocytica lymphohistiocytosis. J Pediatr. 162(6):1245-51, 2013
6- Schulert GS, Grom AA: Pathogenesis of macrophage activation syndrome and potential for cytokine- directed therapies. Annu Rev Med. 66:145-59, 2015

MeSS Score

Well-differentiated thyroid carcinoma is the third most common solid malignancy in children with a 2% rise in a year incidence. It is more commonly seen in females in their adolescent years with the papillary histologic variant and more than 40% of cases having cervical node disease upon presentation. Surgery (total thyroidectomy) is the mainstay method of management producing along with the use of postoperative radioiodine therapy an excellent long term survival in most cases. Many clinical variables have been utilized to predict prognosis with varying amounts of scientific certainty such as histological tumor type, age, gender, extent of primary-site disease, presence or absence of nodal disease, extent of thyroidectomy, and the use of radioactive iodine ablation. Several studies have found male gender, increasing primary  disease site extension, and follicular histologic subtype all had negative prognostic influence on overall survival. The adult prognostic scales of thyroid carcinoma utilized such as AGES (age, grade, extent of disease, size), AMES (age, metastasis, extent of disease, size), MACIS (metastasis, age at presentation, completeness of surgical resection, invasion, size),  and TNM (tumor, node, metastasis) are not reliable in children. Instead recent evidence has found in children that the presence of distant metastasis, tumor size and gender are independent predictors of mortality at the time of diagnosis. Based on these findings, a final prognostic scale was created and defined as distant metastasis (Me), larger primary tumor size (S), and male sex (S) (MeSS) = +5 (if distant metastasis present), +2 (if primary tumor size > 4 cm), and +3 (if male gender). MeSS score < 2 has a 0% mortality, 2-7 moderate risk (2.7% mortality) and >7 has high risk (23% mortality).

1- Hogan AR, Zhuge Y, Perez EA, Koniaris LG, Lew JI, Sola JE: Pediatric thyroid carcinoma: incidence and outcomes in 1753 patients. J Surg Res. 156(1):167-72, 2009
2- Haigh PI, Urbach DR, Rotstein LE: AMES prognostic index and extent of thyroidectomy for well-differentiated thyroid cancer in the United States. Surgery. 136(3):609-16, 2004
3- Cushing SL, Palme CE, Audet N, Eski S, Walfish PG, Freeman JL: Prognostic factors in well-differentiated thyroid carcinoma. Laryngoscope. 114(12):2110-5, 2004
4- Shapiro NL, Bhattacharyya N: Population-based outcomes for pediatric thyroid carcinoma. Laryngoscope. 115(2):337-40, 2005
5- Shayota BJ, Pawar SC, Chamberlain RS: MeSS: A novel prognostic scale specific for pediatric well-differentiated thyroid cancer: a population-based, SEER outcomes study. Surgery. 154(3):429-35, 2013
6- Palme CE, Waseem Z, Raza SN, Eski S, Walfish P, Freeman JL: Management and outcome of recurrent well-differentiated thyroid carcinoma. Arch Otolaryngol Head Neck Surg. 130(7):819-24, 2004

Lymphoplasmacytic Sclerosing Pancreatitis

Lymphoplasmacytic sclerosing pancreatitis (LPSP) is a very rare cause of chronic pancreatitis occurring in the pediatric age group that can mimic a malignant pancreatic lesion. LPSP is an autoimmune from of chronic pancreatitis found most commonly in elderly men. It occurs in the absence of gallstone pancreatitis, pancreas divisum, or excess alcohol ingestion. It is a diffuse fibrosing process of the pancreas considered an autoimmune condition associated with inflammatory bowel disease, Sjögren syndrome, primary biliary cirrhosis and atopic conditions. It has been proposed that an autoimmune mechanism against carbonic anhydrase II or lactoferrin and a Th1-type immune response may be involved in LPSP. Histologically it can be classic or intermediate in appearance. Classic LPSP has lymphoplasmacytic infiltration of the pancreas, interstitial fibrosis, periductal inflammation, and periphlebitis, while intermediate LPSP includes patients with at least two of these histological findings. Clinically the patient with LPSP presents with weight loss, jaundice, abdominal pain, elevated CEA and CA-19 levels similar to patients with pancreatic cancer. On imaging (CT, MRI, ERCP) the mass is usually in the head of the pancreas with a more diffuse appearance and irregular narrowing of the pancreatic dust as compared to the more discrete form of mass of an adenocarcinoma. LPSP produces high serum levels of IgG4, a rare gamma globulin subclass, that is not elevated in pancreatic cancer. FNA biopsy of the lesion can bring conflicting results. FNA histopathological examinations of the pancreas show fibrosis and pronounced infiltration of cells, mainly lymphocytes and plasmacytes. Initial management of LPSP should consist of systemic steroid therapy which can cause remission of the mass to normal level when follow-up with imaging studies in most cases.

1- Okazaki K, Uchida K, Matsushita M, Takaoka M: How to diagnose autoimmune pancreatitis by the revised Japanese clinical criteria. J Gastroenterol. 42 Suppl 18:32-8, 2007
2- Klimstra DS, Adsay NV: Lymphoplasmacytic sclerosing (autoimmune) pancreatitis. Semin Diagn Pathol. 21(4):237-46, 2004
3- Hardacre JM, Iacobuzio-Donahue CA, Sohn TA, Abraham SC, Yeo CJ, Lillemoe KD, Choti MA, Campbell KA, Schulick RD, Hruban RH, Cameron JL, Leach SD: Results of pancreaticoduodenectomy for lymphoplasmacytic sclerosing pancreatitis. Ann Surg. 237(6):853-8, 2003
4- de Castro SM, de Nes LC, Nio CY, Velseboer DC, ten Kate FJ, Busch OR, van Gulik TM, Gouma DJ: Incidence and characteristics of chronic and lymphoplasmacytic sclerosing pancreatitis in patients scheduled to undergo a pancreatoduodenectomy. HPB (Oxford). 12(1):15-21, 2010
5- Wang G, Zhu H, Yuan CX, Gao Y, Li J, Xue DB, Sun B: Lymphoplasmacytic sclerosing pancreatitis with obstructive jaundice: a case report and review of the literature. Onkologie. 32(8-9):506-8, 2009
6- Bartholomew SV, Zigman A, Sheppard B: Lymphoplasmacytic sclerosing pancreatitis presenting as a pancreatic head mass in a child: case report and management recommendations. J Pediatr Surg. 41(5):e23-5, 2006

PSU Volume 45 No 06 DECEMBER 2015

Hair Tourniquet Syndrome

Hair tourniquet syndrome (HTS) refers to hair or other thread fiber becoming tightly wrapped circumferentially around an appendage of the child causing strangulation and compromised blood flow. Appendages affected in such syndrome include in order of preference toe, finger, uvula, tongue or genital structure (penis and clitoris). The third toe is the most frequent affected digit. Erythema and circumferential cut with distal edema is typical compromising the distal venous and lymphatic drainage of the appendage involved (compartment syndrome). Further edema leads to interruption of arterial blood supply causing ischemic injury, tissue necrosis and autoamputation. This process can occur over hours to weeks. It is important to distinguish HTS from congenital constriction band syndrome (amniotic band syndrome, Streeter's dysplasia), the latter being a rare congenital condition that is associated with other musculoskeletal disorders. The typical case is a 5 month old infant. Males and female are equally affected. Hairs are suppled and stretched easily when wet and contract when dry, and the circular configuration results in hydrogen-bonds giving a firmer hold. The high tensile strength of hair makes it an effective tourniquet. Management consists of release of the strangulation under either local or general anesthesia urgently. The constricting hair or fiber may be mechanically removed or depilatory agents may be used. A short longitudinal incision (peritendinous) placed perpendicular to the constricting band can be dorsally made in the digit all the way to the bone to facilitate a definitive release since severe inflammation can deeply bury the offending agent. The strand of hair will be then be seen and removed completely. Antibiotic coverage should be provided as appropriate.

1- Serour F, Gorenstein A: Treatment of the toe tourniquet syndrome in infants. Pediatr Surg Int. 19(8):598-600, 2003
2- Badawy H, Soliman A, Ouf A, Hammad A, Orabi S, Hanno A: Progressive hair coil penile tourniquet syndrome: multicenter experience with 25  cases. J Pediatr Surg. 45(7):1514-8., 2010
3- O'Gorman A, Ratnapalan S: Hair tourniquet management. Pediatr Emerg Care. 27(3):203-4, 2011
4- Bannier MA, Miedema CJ: Hair tourniquet syndrome. Eur J Pediatr. 172(2):277, 2013
5- Bean JF, Hebal F, Hunter CJ: A single center retrospective review of hair tourniquet syndrome and a proposed treatment algorithm. J Pediatr Surg. 50(9):1583-5, 2015
6- Kuiper JW, de Korte N: Hair thread tourniquet syndrome in a toe of an 18 mo old girl. World J Clin Cases. 3(4):368-70, 2015

Blunt Cardiac Injury

Injury to the chest can cause trauma to the heart. Blunt cardiac injury (BCI) is rare in children. Mechanisms associated with such trauma include thrusting of the heart against the chest wall as a direct result of sudden acceleration or deceleration, compression of the heart between the sternum and the vertebral column, and a sudden violent increase in intrathoracic pressures associated with crushing chest or abdominal injuries. Most cases occur after motor vehicle accidents. Most (95%) BCI is cardiac contusion followed by valvular dysfunction and ventricular septal defects. Other sequelae such as hemopericardium, pericardial effusion, arrhythmias, cardiac aneurysm, and myocardial rupture may only occur after some delay emphasizing the need for observation and serial evaluation. Free wall rupture is lethal. Chest pain is present in 50% of neurologically responsive children. The most useful investigations in suspected cases of myocardial contusion are serial cardiac troponins, an initial and repeat ECG, echocardiography and arrhythmia monitoring. Troponin 1 level is a sensitive and more specific measure than CK-MB. They become elevated within hours of injury and remain elevated for 4-7 days. Levels above 8.0 ng/ml are associated with a fatal outcome. Troponin is also very useful in cases of non-accidental chest trauma, such as child abuse. In general, patients with myocardial contusion have a favorable outcome. Normalization of cardiac troponin can help to risk-stratify patients. VSD after BCI is also very rare, occurs more commonly in the muscular septum followed by the membranous portion due to initial damage of the nutrient vessels supplying the septum. Conduction anomalies, such as RBB and AV block, along with arrhythmias such as tachycardia and extrasystole or ventricular fibrillation can occur depending on  damage. Angiography is the gold standard for confirming coronary involvement.

1- Karpas A, Yen K, Sell LL, Frommelt PC: Severe blunt cardiac injury in an infant: a case of child abuse. J Trauma. 52(4):759-64, 2002
2- Milligan J, Potts JE, Human DG, Sanatani S: The protean manifestations of blunt cardiac trauma in children. Pediatr Emerg Care. 2005 May;21(5):312-7
3- Huguet M, Tobon-Gomez C, Bijnens BH, Frangi AF, Petit M: Cardiac injuries in blunt chest trauma. J Cardiovasc Magn Reson. 17;11:35, 2009
4- Bennett BL, Mahabee-Gittens M, Chua MS, Hirsch R: Elevated cardiac troponin I level in cases of thoracic nonaccidental trauma. Pediatr Emerg Care. 27(10):941-4, 2011
5- Acker SN, Stewart CL, Roosevelt GE, Partrick DA, Moore EE, Bensard DD: When is it safe to forgo abdominal CT in blunt-injured children? Surgery. 158(2):408-12, 2015
6- Skinner DL, den Hollander D, Laing GL, Rodseth RN, Muckart DJ: Severe blunt thoracic trauma: differences between adults and children in a level  I trauma centre. S Afr Med J. 105(1):47-51, 2015

VATS: Spontaneous Pneumothorax

Spontaneous pneumothorax (SP) in children is usually primary and caused by rupture of a subpleural bleb or bulla in adolescent, tall and thin patients. Secondary SP occurs in children with underlying lung disease such as asthma, cystic fibrosis, emphysema or connective tissue disorder. The goals of treatment of SP consist of lung re-expansion and avoidance of recurrence. Initial management with needle aspiration or tube thoracostomy drainage have a high failure and recurrent rate. Instillation of chemicals to sclerose the pleuras through the chest tube have also a significant rate of recurrence. A wide variety of chemical sclerosing agents have been introduced into the pleural space to achieve pleural symphysis. The most popular agents have been talc, tetracycline, and silver nitrate, which are known to cause aseptic inflammation leading to dense adhesions. Talc has the lowest recurrence rate of all chemical agents, while tetracyclines are the most innocuous. During the last decade video-assisted thoracoscopic surgery (VATS) has replaced open thoracotomy in the management of spontaneous pneumothorax. Using VATS the surgeon can resect the offending bleb or bulla and produce chemical or mechanical pleurodesis with less pain, less respiratory dysfunction, less hospital stay and better cosmetic results. VATS with blebectomy can be used during the first SP episode if there is persistent chest tube air leak, a contralateral SP develops in a known patient or he has respiratory insufficiency. The two most common complications after VATS for SP are prolonged air leak and bleeding. Prolonged leak is associated with an underlying lung disease such as asthma, cystic fibrosis or emphysema. Postoperative air leak from the staple lines probably accounts for most of the need for postop chest tube drainage. Prolonged air leak is managed with continuous chest tube suction and further chemical pleurodesis. Both factors increase hospital stay. Bleeding might occur after dense adhesions of previous recurrent episodes of SP or after intercostal artery injury from the trocars. Children with SP should avoid activities that put additional strain on the lungs including scuba diving, airplane flight and playing wind musical instruments. Late recurrence is related to formation of new bullae.

1- Ozcan C, McGahren ED, Rodgers BM: Thoracoscopic Treatment of Spontaneous Pneumothorax in Children. J Pediatr Surg. 38(10): 1459-1464, 2003
2- Bialas RC, Weiner TM, Phillips JD: Video-assisted thoracic surgery for primary spontaneous pneumothorax in children: is there an optimal technique? J Pediatr Surg. 43(12):2151-5, 2008
3- Lee LP, Lai MH, Chiu WK, Leung MW, Liu KK, Chan HB: Management of primary spontaneous pneumothorax in Chinese children. Hong Kong Med J. 16(2):94-100, 2010
4- Seguier-Lipszyc E, Elizur A, Klin B, Vaiman M, Lotan G: Management of primary spontaneous pneumothorax in children. Clin Pediatr (Phila). 50(9):797-802, 2011
5- Choi SY, Kim YH, Jo KH, Kim CK, Park JK, Cho DG, Jeong SC, Jeon HW, Park CB: Video-assisted thoracoscopic surgery for primary spontaneous pneumothorax in children. Pediatr Surg Int. 29(5):505-9, 2013
6- Lopez ME, Fallon SC, Lee TC, Rodriguez JR, Brandt ML, Mazziotti MV: Management of the pediatric spontaneous pneumothorax: is primary surgery the treatment of choice? Am J Surg. 208(4):571-6, 2014
7- Noh D, Lee S, Haam SJ, Paik HC, Lee DY: Recurrence of primary spontaneous pneumothorax in young adults and children. Interact Cardiovasc Thorac Surg. 21(2):195-9, 2015

Journal Club