PSU Volume 49 No 01 JULY 2017

Refeeding Syndrome

Refeeding syndrome (RFS) is a potentially lethal condition defined as severe electrolyte and fluid imbalanced associated with metabolic abnormalities in malnourished patients undergoing refeeding orally, enterally or parenterally. Children at risk for developing RFS include those with chronic malnutrition, protein malnutrition, marasmus, cancer, alcoholism, following prolonged starvation, massive weight loss, prolonged IV repletion and anorexia nervosa. Predominant features of RFS include severe hypophosphatemia, altered glucose metabolism, hypokalemia, hypomagnesemia and thiamine deficiency leading o serious cardiac, pulmonary, hematological and central nervous system complications.  RFS can occur after five to ten days of starvation. Fats become the predominant source of energy. Insulin is suppressed and glucagon increases. Intracellular and extracellular ions are chronically depleted including sodium, potassium, magnesium and phosphorus. When normal nutrition is resumed insulin secretion increases and glycogen, fat and protein synthesis begins. The decreased phosphate depletes ATP and other phosphate-based molecules like 2,3-DPG intracellularly leading to poor oxygen delivery and electrolyte abnormalities from movement of ions into the cell. The patient with RFS can develop arrhythmias and cardiac dysfunction, gastrointestinal complaint, muscle weakness, myalgia, dyspnea, respiratory failure, hematologic disturbances and even death. Labs performed reveal hyponatremia, hypokalemia, hypomagnesemia, hypophosphatemia, hyperglycemia and trace and vitamins' deficiencies. Management of RFS includes slow nutritional supplementation approximately 20 Kcal/kg/day or 25% of calories requirements daily advancing three to five days for the total value with administration of deficient nutrients, trace elements, mineral and vitamins. RFS is most commonly reported in those receiving TPN but can occur also in patients who received intravenous saline-dextrose, tube feeding or an oral diet.


References:
1- Crook MA: Refeeding syndrome: problems with definition and management. Nutrition. 30(11-12): 1448-55, 2014
2- Boateng AA, Sriram K, Meguid MM, Crook M: Refeeding syndrome: Treatment considerations based on collective analysis of literature case reports. Nutrition 26(2): 156-67, 2010
3- Al Sharkawy I, Ramadan D, El-Tantawy A: 'Refeeding syndrome' in a Kuwait child: clinical diagnosis and management. Med Princ Pract. 19(3):240-3, 2010
4- Viana LdA, Burgos MG, Silva RdA: Refeeding Syndrome: Clinical and Nutritional Relevance.  ABCD Arq Bras Cir Dig 25(1): 56-59, 2012
5- Agarwal J, Poddar U, Yachha SK, Srivastava A: Refeeding syndrome in children in developing countries who have celiac disease. J Pediatr Gastroenterol Nutr. 54(4):521-4, 2012
6- Pulcini CD, Zettle S, Srinath A: Refeeding Syndrome. Pediatr Rev. 37(12):516-523, 2016

Tap Water Iontophoresis

Idiopathic (primary) excessive hyperhydrosis is excessive sweating without known etiology. It usually occurs in the palmar, plantar, axilla, facial or cervical region usually bilateral causing in the child's social, psychological, emotional and professional difficulty. Hyperhydrosis usually starts in childhood and may continue for the rest of the life, does not occur during sleep for it is normally stimulated by emotion and stress. Management of hyperhydrosis includes aluminum chloride antiperspirants, systemic anticholinergic agents, injection of botulinum toxin A (Botox) and when is very severe surgical thoracic sympathectomy. Recently tap water or normal saline iontophoresis (TWI) has been found to be safe, effective and inexpensive treatment for idiopathic hyperhydrosis. Iontophoresis is an electrical treatment that includes absorption of a drug or chemical in the form of ions on the skin. It is used in local anesthesia, antibacterial modality, neurogenic pain relief, edema, chronic ulcer repair, skin fungi infection and hyperhydrosis. Pore obstruction of sweat ducts secondary to hyperkeratinization, impairment of the electrochemical gradient of sweat and biofeedback mechanism may be involved in hyperhydrosis following iontophoresis using tap water or normal saline. Iontophoresis is an effective treatment with an immediate result possibly due to mechanical or electrochemical occlusion of sweat ducts which can last for at least four weeks. The efficiency of normal saline iontophoresis has been found to be greater and 70% more efficient than that of tap water. Burning and pin pricking sensations are very common and may be felt by all treated patient. Dryness, cracking, erythema and vesiculation which are transient have also been reported after iontophoresis. Pregnant women, people with pacemaker or metal implants, cardiac condition or epilepsy are contraindications for use of iontophoresis therapy. TWI should be offered before considering more invasive surgical procedures.

References:
1- Gunathilake R: Tap water iontophoresis versus glycopyrrolate iontophoresis. Aust Fam Physician. 42(7):441, 2013
2- Dogruk Kacar S, Ozuguz P, Eroglu S, Polat S, Karaca S: Treatment of primary hyperhidrosis with tap water iontophoresis in paediatric patients: a retrospective analysis. Cutan Ocul Toxicol. 33(4):313-6, 2014
3- Yaghobi Z, Goljarian S, Oskouei AE: Comparison of tap water and normal saline iontophoresis in idiopathic hyperhidrosis: a case report. J Phys Ther Sci. 26(8):1313-5, 2014
4- Nagar R, Sengar SS: A Simple User-made Iontophoresis Device for Palmoplantar Hyperhidrosis.  J Cutan Aesthet Surg. 9(1):32-3, 2016
5- Dagash H, McCaffrey S, Mellor K, Roycroft A, Helbling I: Tap water iontophoresis in the treatment of pediatric hyperhidrosis.  J Pediatr Surg. 52(2):309-312, 2017

Transverse Testicular Ectopia

Transverse (or crossed) testicular ectopia (TTE) is a very rare congenital condition in which both testes migrate toward the same hemiscrotum through the same inguinal canal. Theories that explain this rare behavior of both testis residing in the same hemiscrotum include both testis being derived from the same germinal ridge through duplication of the gonadal primordium, mechanical effect of persistent Müllerian duct structures preventing testicular decent and causing both testicles to descend toward the same scrotum and defective gubernacular formation. The ectopic testis may lie in the opposite hemiscrotum, inguinal canal or at the deep inguinal ring. TTE is usually associated with other anomalies such as persistent Müllerian duct syndrome, true hermaphroditism, inguinal hernia (most common association), renal agenesis and scrotal anomalies. The diagnosis of TTE is made on clinical examination and confirmed using scrotal ultrasonography, CT-Scan, MRI or laparoscopy. The patient usually present with uniform symptoms of an inguinal hernia on one side and an impalpable testis on the other side. Each testis has a corresponding spermatic cord but in most cases the two cords fuse to form an inseparable thick-walled structure several centimeters proximal to the testis. Each testis has its own blood supply. Both testes share a single patent processus vaginalis. TTE can be reclassified depending if it had a hernia (type 1), is accompanied with persistent Müllerian structures (type 2), or associated with other disorders mentioned above (type 3). Surgical management consists of either trans-septal orchiopexy or extraperitoneal transposition orchiopexy. Infertility and risk of seminoma are known long-term complications needing close follow-up. 

References:
1- Dahal P, Koirala R, Subedi N: Transverse testicular ectopia: a rare association with inguinal hernia. J Surg Case Rep. 2014(10), 2014
2- Kumar L, Garg P, Rao A, Kumar A: Transverse testicular ectopia with bilateral pyocoele: case report and brief review of literature. J Surg Tech Case Rep. 6(2):55-7, 2014
3- Kamble RS, Gupta RK, Gupta AR, Kothari PR, Dikshit KV, Kesan KK: Laparoscopic management of transverse testicular ectopia with persistent Müllerian duct syndrome. J Minim Access Surg. 11(3):213-5, 2015
4- Punwani VV, Wong JS, Lai CY, Chia JC, Hutson JM: Testicular ectopia: Why does it happen and what do we do?  J Pediatr Surg. 2016 Dec 21. pii: S0022-3468(16)30648-0. doi: 10.1016/j.jpedsurg.2016.12.009.
5- Kajal P, Rattan KN, Bhutani N, Sangwan V: Transverse testicular ectopia with scrotal hypospadias but without inguinal hernia - Case report of a rare association. Int J Surg Case Rep. 31:167-169, 2017


PSU Volume 49 No 02 AUGUST 2017

Gastrojejunostomy Tube

Gastrojejunostomy tube (GJT) insertion is a common procedure performed to provide postpyloric enteral nutrition in children and adults. GJT is an alternative to gastrostomy tubes when the stomach cannot be fed directly due to history of gastroparesis, gastroesophageal reflux, failed fundoplication, aspiration pneumonia or small capacity stomach (microgastria). GJT are placed via open laparotomy, endoscopic-assisted laparoscopy or fluoroscopic technique after a previous gastrostoma. Open, endoscopic and laparoscopic technique includes the used if intraoperative fluoroscopy. GJT tip placement should be placed distal to the third portion of the duodenum. All cases should have fluoroscopic contrast studies during insertion to demonstrate that no bowel perforation has occurred. Complications associated with the use of GJT include the need for tube replacement, peristomal granulation or leakage, recurrent symptoms of gastroesophageal reflux, intussusception and intestinal perforation. The most frequent reported complications are the need for tube replacement due to mechanical failure from tube fracture or balloon rupture, tube obstruction from clogging or tube displacement from complete removal or distal migration. Almost 75% of children require a return to the operating room for GJT replacement with a mean of two replacements per year. Children weighting less than 6 kilograms or younger than six months of age are at a higher risk of suffering an intestinal perforation with the use of GJT. The complication with the greatest potential morbidity is that related to intestinal perforation which can lead to death. The perforation occurs nears the ligament of Treitz and usually occurs within the first 30 days after insertion. This occurs due to the relative rigidity of the jejunal extension of the GJT which exerts radial pressure on the duodenojejunal junction in the area of the ligament of Treitz where the bowel makes a sharp turn. The smaller the child the smaller the bowel diameter and size. The tip causes pressure necrosis leading to perforation.

References:
1- Jaskolka D, Brown N, Cohen E, Mounstephen W, Connolly B: Evaluating the implementation of a quality improvement initiative: weekend gastrojejunostomy tube maintenance service in a tertiary pediatric center. Can Assoc Radiol J. 64(3):229-35, 2013
2- Crowley JJ, Hogan MJ, Towbin RB: Quality improvement guidelines for pediatric gastrostomy and gastrojejunostomy tube placement.  J Vasc Interv Radiol. 25(12):1983-91, 2014
3- Richards MK, Li CI, Foti JL, Leu MG, Wahbeh GT, Shaw D, Libby AK, Melzer L, Goldin AB: Resource utilization after implementing a hospital-wide standardized feeding tube placement pathway. J Pediatr Surg. 51(10):1674-9, 2016
4- Demehri FR, Simha S, Herrman E, Jarboe MD, Geiger JD, Teitelbaum DH, Gadepalli SK: Analysis of risk factors contributing to morbidity from gastrojejunostomy feeding tubes in children. J Pediatr Surg. 51(6):1005-9, 2016
5- Morse J, Baird R, Muchantef K, Levesque D, Morinville V, Puligandla PS: Gastrojejunostomy tube complications - A single center experience and systematic  review. J Pediatr Surg. 52(5):726-733, 2017
6- Onwubiko C, Weil BR, Bairdain S, Hall AM, Perkins JM, Thangarajah H, McSweeney ME, Smithers CJ: Primary laparoscopic gastrojejunostomy tubes as a feeding modality in the pediatric population. J Pediatr Surg. pii: S0022-3468(17)30333-0. doi: 10.1016/j.jpedsurg.2017.05.015, 2017

Sutureless Gastroschisis

Gastroschisis is a congenital abdominal wall defect to the right of the umbilical cord resulting in uncovered bowel protruding. The exposed viscera bathing in the amniotic fluid create a serositis with a foreshortened edematous bowel. Gastroschisis is a newborn emergency that should be managed by reducing progressively the exposed abdominal content and closing the abdominal wall defect surgically. Closure depends on the degree of viscero-abdominal disproportion found upon birth, the condition of the bowel and the presence of an associated intestinal atresia. Management can consist of closure using a silo-spring loaded silo where the bowel is housed and progressively reduced over the next few days then followed by fascial closure or primary closure if the defect can be closed initially. The advent of staged closure allowed for a reduction of pulmonary, renal and ischemic complications related to increased intra-abdominal pressure caused by aggressive primary reduction. Sutureless umbilical technique closure refers to the use of remnant umbilical cord as a biological dressing reinforce with a non-adherent synthetic material such as Tegaderm dressing on top. The procedure may be performed bedside in the NICU with minimal sedation. Adhesive dressing are changed over the umbilical cord closure every other day for two weeks and the wound quickly epithelizes during this time. The proposed advantages of this technique include lower total cost, better cosmetic outcome and transfer of the procedure from the operating room to bedside. Sutureless closure of gastroschisis reduces the time to extubation independently of all variables due to the impact of this approach on intraabdominal pressure, the secondary improvement of ventilation mechanics and reduced need for narcotics and sedation. Time to feeding is not altered by either sutureless or surgical closure techniques. Almost all cases of sutureless technique closures develop an umbilical hernia. Most resolve spontaneously and do not require formal repair. Sutureless closure of uncomplicated gastroschisis is a safe technique that reduces need of intubation and provides excellent cosmetic results.

References:
1- Sandler A, Lawrence J, Meehan J, Phearman L, Soper R: A "plastic" sutureless abdominal wall closure in gastroschisis. J Pediatr Surg. 39(5):738-41, 2004
2- Weinsheimer RL, Yanchar NL, Bouchard SB, Kim PK, Laberge JM, Skarsgard ED, Lee SK, McMillan D, von Dadelszen P; Canadian Pediatric Surgery Network: Gastroschisis closure--does method really matter? J Pediatr Surg. 43(5):874-8, 2008
3- Riboh J, Abrajano CT, Garber K, Hartman G, Butler MA, Albanese CT, Sylvester KG, Dutta S: Outcomes of sutureless gastroschisis closure. J Pediatr Surg. ;44(10):1947-51, 2009
4- Choi WW, McBride CA, Bourke C, Borzi P, Choo K, Walker R, Nguyen T, Davies M,  Donovan T, Cartwright D, Kimble RM: Long-term review of sutureless ward reduction in neonates with gastroschisis in the neonatal unit. J Pediatr Surg. 47(8):1516-20, 2012
5- Chesley PM, Ledbetter DJ, Meehan JJ, Oron AP, Javid PJ: Contemporary trends in the use of primary repair for gastroschisis in surgical infants. Am J Surg. 209(5):901-5, 2015
6- Zajac A, Bogusz B, Soltysiak P, Tomasik P, Wolnicki M, Wedrychowicz A, Wojciechowski P, Gorecki W: Cosmetic Outcomes of Sutureless Closure in Gastroschisis. Eur J Pediatr Surg. 26(6):537-541, 2016

Fetal Ovarian Torsion

Fetal ovarian cysts are the most common abdominal masses detected with an increased incidence since the advent of prenatal ultrasonography. Fetal ovarian cysts are usually seen toward the end of the second trimester of pregnancy. Stimulation of the fetal ovary by placental and maternal chorionic gonadotropin hormones leads to development of ovarian cysts. Once diagnosed prenatally, ovarian cysts should be monitored carefully since hemorrhage, torsion and rupture with loss of ovarian tissue can ensue. Postnatally the baby should have an US to determine of the cyst is simple or complex. Simple cysts also referred as follicular cysts are usually unilocular and completely anechoic on US.  Most simple cysts below the average size of 4 cm can be observed for spontaneous resolution. Those beyond 4 cm are at risk to developed torsion and should be aspirated either open, percutaneously or laparoscopically. Complex cysts which are more concerning can have echogenic wall, internal septa, fluid-debris level or a blood clot. Most (86%) postnatal hemorrhagic ovarian cysts have sustained torsion. Ovarian torsion occurs from either partial or complete twist of the ovary and fallopian tube. Conservative surgery is recommended in the first days of life if there are US signs of hemorrhage within the cyst during birth or immediately after birth. When torsion of a cyst occurs its size increases rapidly and US features change to complex. Complex cysts will have debris, internal septa or hemorrhage. Color spectral Doppler US can be used to evaluate ovarian vascularity. Unilaterally enlarged ovary with peripheral cysts containing fluid-debris level are highly suspicious for ovarian torsion and hemorrhagic infarction. Absence of blood flow  is indicative of torsion. If the US findings are equivocal, an MRI can be performed for further characterization. Most torsion of fetal ovarian cysts occurs prenatally. A calcified abdominal mass, with or without wandering, can be an autoamputated ovary.

References:
1- Galinier P, Carfagna L, Jurisic M, et al: Fetal ovarian cysts management and ovarian prognosis: a report of 82 cases. J Pediatr Surg 43: 2004-2009, 2008
2- Koike Y, Inoue M, Uchida K, Kawamoto A, Yasuda H, Okugawa Y, Otake K, Kusunoki M: Ovarian autoamputation in a neonate: a case report with literature review. Pediatr Surg Int. 25(7):655-8, 2009
3- Sheth R, Hoelzer D, Scattergood E, Germaine P: In utero fetal ovarian torsion with imaging findings on ultrasound and MRI. Case Rep Radiol. 2012;2012:151020
4- Levit A, Voci SL: In utero fetal ovarian torsion. Ultrasound Q. 30(3):249-50, 2014
5- Ozcan HN, Balci S, Ekinci S, Gunes A, Oguz B, Ciftci AO, Haliloglu M: Imaging Findings of Fetal-Neonatal Ovarian Cysts Complicated With Ovarian Torsion and Autoamputation. AJR Am J Roentgenol. 205(1):185-9, 2015
6-Kim HS, Yoo SY, Cha MJ, Kim JH, Jeon TY, Kim WK: Diagnosis of neonatal ovarian torsion: Emphasis on prenatal and postnatal sonographic findings.  J Clin Ultrasound. 44(5):290-7, 2016


PSU Volume 49 NO 03 SEPTEMBER 2017

Schatzki Ring

Schatzki ring (SR) is a thin diaphragm type of circumferential fold of mucosa that protrude into the lumen of the distal esophagus. It usually measures between one and 2 mm in thickness and is located in the gastroesophageal squamocolumnar junction. Schatzki ring is a very rare condition identified in the pediatric population. SR are found in 6-14% of routine barium esophagograms. Though SR are asymptomatic, they are considered to be the most common cause of episodic dysphagia for solids and food impaction in adults. SR pathogenesis has been associated with congenital, anatomical (hiatal hernia), and inflammatory conditions such as that associated with gastroesophageal reflux or eosinophilic esophagitis. Because SR is located at the squamous columnar junction, the most widely accepted theory of pathogenesis is that it is part of the spectrum of chronic mucosal inflammation resulting from acid reflux which is not always found to be true. The level of esophagitis and positive pH monitoring found in patients with SR is less than 50% of the time. Patients with SR have a normal lower esophageal sphincter pressure. It is suggested that there are two types of SR depending on esophagoscopy findings: a more common intermittent type and a less common fixed one. The intermittent type is produced by mucosal plication and is the type seen associated with eosinophilic esophagitis, while patients with peptic esophagitis may have the fixed type. Most cases of SR are males and the most common clinical presentation is dysphagia and food impaction with a mean duration of four years prior to diagnosis. SC is also associated with hiatal hernia, esophageal web and esophageal diverticula. Diagnosis of SR entail endoscopy with biopsy, esophagogram and manometry studies. Management consist of observation if asymptomatic, proton pump inhibitors if reflux is identified, or esophageal dilatation (balloon or bougie) if fixed, symptomatic and narrow.    


References:
1- Nurko S, Teitelbaum JE, Husain K, Buonomo C, Fox VL, Antonioli D, Fortunato C, Badizadegan K, Furuta GT: Association of Schatzki ring with eosinophilic esophagitis in children. J Pediatr Gastroenterol Nutr. 38(4):436-41, 2004
2- Muller M(1), Gockel I, Hedwig P, Eckardt AJ, Kuhr K, Konig J, Eckardt VF: Is the Schatzki ring a unique esophageal entity? World J Gastroenterol. 17(23):2838-43, 2011
3- Towbin AJ, Diniz LO: Schatzki ring in pediatric and young adult patients. Pediatr Radiol. 42(12):1437-40, 2012
4- Muller M, Eckardt AJ, Fisseler-Eckhoff A, Haas S, Gockel I, Wehrmann T: Endoscopic findings in patients with Schatzki rings: evidence for an association with eosinophilic esophagitis. World J Gastroenterol. 18(47):6960-6, 2012
5- Patel B, Han E, Swan K: Richard Schatzki: a familiar ring. AJR Am J Roentgenol. 201(5):W678-82, 2013
6- Ashraf HH, Palmer J, Dalton HR, Waters C, Luff T, Strugnell M,  Murray IA: Can patients determine the level of their dysphagia? World J Gastroenterol. 23(6):1038-1043, 2017

Postsurgical Hypoparathyroidism

Hypoparathyroidism (HypoPT) is an uncommon disorder characterized by hypocalcemia and hyperphosphatemia due to insufficient secretion of parathyroid hormone. By far the most common cause of hypoparathyroidism is postsurgical followed by autoimmune disorders, genetic causes, metastatic disease, iron overload or ionizing radiation. Neck surgery for thyroid cancer, goiter (toxic and nontoxic) and hyperparathyroidism is the most common cause of acquired hypoparathyroidism responsible for 75% of all cases. Almost 7% of surgical procedures related to the thyroid or parathyroid gland results in hypoparathyroidism, with 75% being transient and 25% chronic (> 6 months). Acquired HypoPT occurs after removal, irreversible damage or vascular ischemia of the parathyroid glands. Parathyroid hormone enhances renal tubular calcium reabsorption and increases phosphate excretion. Lack of parathyroid hormone is associated with an increase renal calcium excretion and hyperphosphatemia. Morbidity associated with HypoPT includes neuromuscular excitability (tetany), renal insufficiency, kidney stones, posterior subcapsular cataracts, intracerebral calcifications, reduced skeletal remodeling, alteration in mood, depression, bipolar disorders and increase risk of infection due to altered immune response. Risk of cardiovascular disease, fractures, spinal stenosis and malignant disease are not increased in patients with HypoPT. Management of hypoparathyroidism with calcium and active vitamin D causes an increase in serum calcium and relief of classical symptoms of hypocalcemia. The main problem is compliance with calcium and vitamin D long-term ingestion. The intake of large amounts of soft drinks containing high amounts of phosphoric acid reduces calcium absorption efficiency despite high doses of calcium therapy. Overall, mortality is not increased with hypoparathyroidism. Successful living-donor parathyroid allotransplantation for postsurgical HypoPT represents an innovative therapeutic strategy that could provide definitive management in which the disease is refractory to therapy.


References:
1- Khan MI, Waguespack SG, Hu MI: Medical management of postsurgical hypoparathyroidism. Endocr Pract. 17 Suppl 1:18-25, 2011
2- Underbjerg L, Sikjaer T, Mosekilde L, Rejnmark L: Postsurgical hypoparathyroidism--risk of fractures, psychiatric diseases, cancer, cataract, and infections.  J Bone Miner Res. 29(11):2504-10, 2014
3- Clarke BL, Brown EM, Collins MT, et al: Epidemiology and Diagnosis of Hypoparathyroidism. J Clin Endocrinol Metab. 101(6):2284-99, 2016
4- Agha A, Scherer MN, Moser C, et al: Living-donor parathyroid allotransplantation for therapy-refractory postsurgical persistent hypoparathyroidism in a nontransplant recipient - three year results: a case report. BMC Surg. 16(1):51, 2016
5- Kakava K, Tournis S, Papadakis G, et al: Postsurgical Hypoparathyroidism: A Systematic Review. In Vivo. 30(3):171-9, 2016
6- Guarnotta V, Riela S, Massaro M, et al: The Daily Consumption of Cola Can Determine Hypocalcemia: A Case Report of Postsurgical Hypoparathyroidism-Related Hypocalcemia Refractory to Supplemental Therapy with High Doses of Oral Calcium. Front Endocrinol (Lausanne). 2017 Jan 26;8:7. doi: 10.3389/fendo.2017.00007. eCollection 2017

Pinpoint

Fluorescent cholangiography (FC) is a technique that can be easily performed with intravenous fluorophore injection and intraoperative near-infrared imaging (NIR) to view its dissemination throughout the biliary system. The pinpoint endoscopic fluorescence imaging system (Novadaq) overlays florescent and color images in a synchronous manner enabling the simultaneous visualization of normal color and florescent images. This enables the simultaneous viewing of fluorescent images and surgery can be performed watching the anatomy of the biliary system without the need of performing and intraoperative cholangiogram. The advantage is that this system can help discern the anatomy of the biliary system while removing the sick gallbladder specially in cases where there is chronic inflammation or subacute cholecystitis thus avoiding the dreadful complication of bile duct injury. Indocyanine Green (ICG) is injected intravenously at anesthesia induction permitting  the cystic duct and common bile duct to be clearly imaged while performing the laparoscopic procedure using the Pinpoint system. Pressing a camera-mounted button Pinpoint can display simultaneous video modes to include conventional white light high definition, fluorescence only, and composite NIR-ICG overlay modes. ICG binds to plasma proteins after IV injection, but it remains in the intravascular space for a few minutes. Bound ICG is taken by the liver and excreted unchanged through the biliary system. ICG injection is well tolerated with very rare serious risk of anaphylaxis. Since it contains iodide, it should be used with caution in patients who are allergic to iodide or iodinated imaging agents. NIR-FC provides sensitive and prolonged identification of biliary anatomy and assessment of functional status. Other applications of NIR-ICG fluorescence includes vascular perfusion assessment of myocutaneous flaps, hollow viscus anastomosis and oncologic procedures.


References:
1- Tsutsui N, Yoshida M, Kitajima M, Suzuki Y: Laparoscopic cholecystectomy using the PINPOINT endoscopic fluorescence imaging system with intraoperative fluorescent imaging: A case report.  Int J Surg Case Rep. 2016;21:129-32.
2- Bozzay J, Vicente D, Jessie EM, Rodriguez CJ: Identification of Abnormal Biliary Anatomy Utilizing Real-Time Near-Infrared Cholangiography: A Report of Two Cases. Case Rep Gastrointest Med. 2017;2017:8628206. doi: 10.1155/2017/8628206. Epub 2017 Apr 27.
3- Watanabe M, Murakami M, Ozawa Y, Yoshizawa S, Matsui N, Aoki T: Intraoperative Identification of Colonic Tumor Sites Using a Near-Infrared Fluorescence Endoscopic Imaging System and Indocyanine Green. Dig Surg. 2017 Feb 21. doi: 10.1159/000458450
4- Ryu S, Yoshida M, Hironori O, Tsutsui N, Suzuki N, Ito E, Nakajima K, Yanagisawa S, Kitajima M, Suzuki Y: Intraoperative ICG fluorescence contrast imaging of the main artery watershed area in colorectal cancer surgery: Report of a case. Int J Surg Case Rep. 2016;26:176-8. doi: 10.1016/j.ijscr.2016.06.009. Epub 2016 Jul 29.
5- Kazanowski M, Al Furajii H, Cahill RA: Near-infrared laparoscopic fluorescence for pelvic side wall delta mapping in patients with rectal cancer--'PINPOINT' nodal assessment. Colorectal Dis. Suppl 3:32-5, 2015


PSU Volume 49 NO 04 OCTOBER 2017

Fowler-Stephen Orchidopexy

Undescended testis (UT) is the most common congenital anomaly of the genitalia of newborn males. It is more common in prematurely born infants. It is estimated that 20% of undescended testis are non-palpable. Non-palpable UT might be intraabdominal, canalicular, atrophic or absent. Orchidopexy in UT should be performed before the age of 18 months to avoid damage to spermatogonia. Diagnostic laparoscopy is the gold standard maneuver to determine localization and eventual management of non-palpable UT. Laparoscopy is better than CT-Scan, MRI, gonadal arteriography or venography in localizing the presence, distance and size of a nonpalpable UT. Blind ending vessels without a distal testis determine absence and no further management is required. If vessels and vas enter the internal spermatic ring, the child probably has a canalicular testis or nubbin in either case it should be moved and fixed to the scrotum or removed respectively suing an inguinal incision. Atrophic or hypoplastic testes are removed during the diagnostic procedure. Intraabdominal testes are either brought down to the scrotum and fixed if the vascular pedicle permits such maneuvers (laparoscopic assisted orchidopexy) or staged its descent using Fowler-Stephen technique (FST). Two-stage FST is performed if the testis is at a high position estimated as more than 2 centimeters between the manipulated testis and the internal ring. The technique consists of clamping and dividing the vascular supply to the testis and leaving it alone so that collateral blood supply develops from the vas deferens. FST can be performed one or two staged procedure. The one-stage FST preserved the gubernaculum for additional collateral blood supply. In the second stage of FST orchidopexy is performed six months later descending the testis with vas deferens-based circulation by fixing it to the scrotum using laparoscopy and inguinal surgery. Success rate for one stage FST can approach 70% which are encouraging. The most common complication after performing either FST is testicular atrophy. 

References:
1- Elder JS: Surgical Management of the Undescended Testis: Recent Advances and Controversies. Eur J Pediatr Surg. 26(5):418-426, 2016
2- Hamidi N, Telli O, Bagci U, Esen B, Karagoz MA, Hascicek AM, Soygur T, Burgu B: Outcomes of Laparoscopic Treatment Modalities for Unilateral Non-palpable Testes. Front Pediatr. 2016 Mar 4;4:13. doi: 10.3389/fped.2016.00013. eCollection 2016.
3- Shehata S, Shalaby R, Ismail M, Abouheba M, Elrouby A: Staged laparoscopic traction-orchiopexy for intraabdominal testis (Shehata technique): Stretching the limits for preservation of testicular vasculature. J Pediatr Surg. 51(2):211-5, 2016
4- Nayci A, Bahadir GB, Erdogan C, Taskinlar H: Laparoscopic one-stage Fowler-Stephens orchiopexy preserving gubernaculum. Turk J Urol. 43(1):75-78, 2017
5- Wang CY, Wang Y, Chen XH, Wei XY, Chen F, Zhong M: Efficacy of single-stage and two-stage Fowler-Stephens laparoscopic orchidopexy in the treatment of intraabdominal high testis. Asian J Surg. Apr 12. pii: S1015-9584(16)30154-3. doi:10.1016/j.asjsur.2016.11.008, 2017
6- Alam A, Delto JC, Blachman-Braun R, Wayne G, Mittal AG, Castellan M, Kozakowski K, Labbie A, Gosalbez R: Staged Fowler-Stephens and Single-stage Laparoscopic Orchiopexy for Intra-abdominal Testes: Is There a Difference? A Single Institution Experience. Urology. 101:104-110, 2017

Epiphrenic Esophageal Diverticulum

Epiphrenic esophageal diverticulum (EED) is a rare out pouching of the lumen of the esophagus originating in the distal end of the esophagus typically four to 10 cm above the cardia and usually projecting from the right posterior wall. EED is a false pulsion diverticulum induced by high intraluminal pressure on the lower esophagus. Herniation of the mucosa and submucosa through the muscle layers of the esophageal wall occurs. Only 15-20% of patient who harbor an EED have symptoms. EED predominant symptoms include dysphagia followed by regurgitation, chest pain, heartburn, weight loss and less frequent atypical respiratory symptoms. The diagnosis of EED should be made performing barium swallow, upper endoscopy and manometry. The size of the diverticulum influences the severity of symptoms. The average size of the diverticulum is 5.5 cm and is associated with an esophageal motility disorder such in 60% of all cases, achalasia leading the list followed by nutcracker esophagus and nonspecific esophageal motor disorder. The  etiology of epiphrenic esophageal diverticulum involves an increased lower esophageal pressure and congenital weakness of the esophageal wall associated with a motor esophageal disorder such as achalasia or diffuse esophageal spasms. The management of an EED consists of laparoscopic diverticulectomy using a linear stapler, long myotomy on the contralateral side of the diverticulum extended to the gastric wall and an anterior Dor partial fundoplication. Complications include leakage at the diverticulum stapled base, empyema, paraesophageal hernia, pneumothorax, atelectasis, pleural effusion and bleeding. Most patient improves significantly with the proposed plan of management. 


References:
1- Abdollahimohammad A, Masinaeinezhad N, Firouzkouhi M: Epiphrenic esophageal diverticula.
J Res Med Sci. 19(8):795-7, 2014
2- Gonzalez-Calatayud M, Targarona EM, Balague C, Rodriguez-Luppi C, Martin AB, Trias M: Minimally invasive therapy for epiphrenic diverticula: Systematic review of literature and report of six cases. J Minim Access Surg. 10(4):169-74, 2014
3- Tamura Y, Funaki Y, Adachi K, Noda H, Izawa S, Iida A, Ogasawara N, Miyaji M, Sasaki M, Kasugai K: A Patient with Vigorous Achalasia and Rapid Enlargement of an Epiphrenic Esophageal Diverticulum. Intern Med. 54(13):1609-12, 2015
4- Matsumoto H, Kubota H, Higashida M, Manabe N, Haruma K, Hirai T: Esophageal epiphrenic diverticulum associated with diffuse esophageal spasm. Int J Surg Case Rep. 13:79-83, 2015
5- Bhandarwar AH, Tungenwar PN, Sawakare YM, Wagh AN, Patel CB, Gandhi SS: Large Epiphrenic Diverticula: A Rare Case Presentation. Clin Pract. 5(4):784, 2015
6- Kang DK: Single-port video-assisted thoracoscopic surgery for a huge epiphrenic esophageal diverticulum. J Thorac Dis. 9(1):E10-E13, 2017
7- Brandeis AE, Singhal S, Lee TH, Mittal SK: Surgical management of epiphrenic diverticulum: A single-center experience and brief review of literature. Am J Surg. 2017 Jul 19. pii: S0002-9610(17)30826-7. doi:10.1016/j.amjsurg.2017.06.039.

Icteropyloric Syndrome

Hypertrophied pyloric stenosis (HPS) is the most common surgical cause of vomiting in the first two months of life occurring mostly in males infants. Management consists of open or laparoscopic pyloromyotomy after adequate hydration from persistent vomiting. Along with HPS some infants develop concomitant jaundice, referred as the icteropyloric syndrome (IPS). The prevalence of icteropyloric syndrome ranges from 8-10% of children with HPS. Initially it was thought that jaundice in the setting of HPS was due to dehydration, mechanical obstruction, decreased carbohydrate intake, decreased hepatic perfusion or a combination of such factors. Later it was believed that these children had Gilbert syndrome genotype. Infants with IPS are younger than infants with HPS suggesting a role for physiologic jaundice. Although prolonged physiologic jaundice could represent a manifestation of Gilbert syndrome, it does not persist into the second month of life. Children with IPS have a significant higher serum bicarbonate and lower serum chloride levels than non-jaundice infants due to greater losses of hydrochloride acid. Metabolic stress potentiates the manifestation of jaundice in infants with IPS along with hepatic levels of residual enzyme activity, bilirubin load and duration of fasting. A Gilbert syndrome genotype is found in 45-65% of infants with IPS. The risk of having Gilbert syndrome genotype is four times higher in IPS than HPS suggesting Gilbert syndrome plays a role in infants with IPS due to molecular defects within the gene promoter. Children with IPS should have genetic workup for Gilbert syndrome mutations. 


References:
1- Lippert MM: Pyloric stenosis presenting as severe prolonged jaundice. A case report. S Afr Med J. 69(7):446-7, 1986
2- Etzioni A, Shoshani G, Diamond E, Zinder O, Bar-Maor JA: Unconjugated hyperbilirubinaemia in hypertrophic pyloric stenosis, an enigma. Z Kinderchir. 41(5):272-4, 1986
3- Trioche P, Chalas J, Francoual J, Capel L, Lindenbaum A, Odiavre M, Labrune P: Jaundice with hypertrophic pyloric stenosis as an early manifestation of Gilbert  syndrome. Arch Dis Child. 81(4):301-3, 1999
4- Hua L, Shi D, Bishop PR, Gosche J, May WL, Nowicki MJ: The role of UGT1A1*28 mutation in jaundiced infants with hypertrophic pyloric stenosis. Pediatr Res. 58(5):881-4, 2005
5- Huang IF, Tiao MM, Chiou CC, Shih HH, Hu HH, Ruiz JP: Infantile hypertrophic pyloric stenosis before 3 weeks of age in infants and preterm babies. Pediatr Int. 53(1):18-23, 2011


PSU Volume 49 NO 05 NOVEMBER 2017

Magnamosis

Magnamosis refers to the concept of performing magnetic compressive anastomosis using two magnets that approach each other due to its attracting forces sloughing the tissue in between them and creating a union. Anastomosis between different parts of our body is a fundamental procedure performed in surgery. Most anastomosis are either hand-sewn or made using mechanical staplers device. Recently a magnamosis device was utilized to perform bowel anastomosis in humans. The magnamosis device is a pair of self-centering rare earth neodymium-iron-boron ring magnets encased in a specially-engineered polycarbonate shell. To create an anastomosis a single magnetic (Harrison ring) is placed within the lumen of each segment of intestine where the union is desired. When the two rings are joined the interposed tissue in between is compressed causing necrosis and anastomosis formation. The device then passes through the newly formed anastomosis and leaves no foreign bodies. The patient then passes the device through the rectum with bowel movement. The magnamosis device has been found to create histologically well-formed anastomosis with burst strength comparable or even better that hand-sen or stapled anastomosis. One side of the device has a slightly convex surface whereas the other side is slightly concave. The compressive forces on the bowel wall causes transmural ischemia and necrosis centrally allowing for remodeling of the bowel in the periphery gradually forming a full-thickness anastomosis. The device is passed in the stools seven to 14 days later depending on the motility of the bowel. Patients can be safely discharge home prior to passing the magnets. The concept of magnamosis has also been utilized for the management of rectal atresia, bilioenteric, esophageal, gastroenteric and vascular anastomosis. It has also been used to managed bile ducts strictures. Magnamosis device is a safe and effective means of sutureless full-thickness bowel anastomosis with serosal apposition.


References:
1- Jamshidi R, Stephenson JT, Clay JG, Pichakron KO, Harrison MR: Magnamosis: magnetic compression anastomosis with comparison to suture and staple techniques. J Pediatr Surg. 44(1):222-8, 2009
2- Pichakron KO, Jelin EB, Hirose S, Curran PF, Jamshidi R, Stephenson JT, Fechter R, Strange M, Harrison MR: Magnamosis II: Magnetic compression anastomosis for minimally invasive gastrojejunostomy and jejunojejunostomy. J Am Coll Surg. 212(1):42-9, 2011
3- Wall J, Diana M, Leroy J, Deruijter V, Gonzales KD, Lindner V, Harrison M, Marescaux J: MAGNAMOSIS IV: magnetic compression anastomosis for minimally invasive colorectal surgery. Endoscopy. 45(8):643-8, 2013
4- Russell KW, Rollins MD, Feola GP, Scaife ER: Magnamosis: a novel technique for the management of rectal atresia. BMJ Case Rep. 2014 Aug 5;2014.
5- Xue F, Guo HC, Li JP, Lu JW, Wang HH, Ma F, Liu YX, Lv Y: Choledochojejunostomy with an innovative magnetic compressive anastomosis: How to determine optimal pressure? World J Gastroenterol. 22(7):2326-35, 2016
6- Dorman RM, Vali K, Harmon CM, Zaritzky M, Bass KD: Repair of esophageal atresia with proximal fistula using endoscopic magnetic compression anastomosis (magnamosis) after staged lengthening. Pediatr Surg Int. 32(5):525-8, 2016
7- Graves CE, Co C, Hsi RS, Kwiat D, Imamura-Ching J, Harrison MR, Stoller ML: Magnetic Compression Anastomosis (Magnamosis): First-In-Human Trial. J Am Coll Surg. 2017 Aug 23. pii: S1072-7515(17)31701-5. doi: 10.1016/j.jamcollsurg.2017.07.1062

Granulomatous Lymphadenitis

Granulomatous inflammation of lymph nodes is the second most common finding in histological examination of peripheral lymph nodes. Reactive hyperplasia accounts for the most common finding. Granulomatous disease accounts for almost one-third of biopsies of cervical masses or lymph nodes in children. They are most commonly identified in the head and neck region. The granulomatous response is a generic reaction to the presence of a persistent endogenous or exogenous insoluble irritant characterized by accumulation of macrophages and dependent of the immune system of the host. Non-tuberculous mycobacteria (NTM) is the etiology of most cases of granulomatous lymphadenitis in children. In developed countries Mycobacterium Avium and Intracellulare accounts for most cases of NTM causing granulomatous lymphadenitis. NTM lymphadenitis in immunocompetent children is best managed with complete excision. Other causes of granulomatous lymphadenitis include tuberculosis, sarcoidosis, fungal infections, rheumatoid disease, Cat's scratch disease and foreign body inclusions. NTM are ubiquitous in the environment existing in soil and water (including tap water) and ingestion of contaminated material has been thought to be the principal route of cervicofacial infection in children. Children with NTM granulomatous lymphadenitis are commonly less than five years in age and more likely have multiple lymph nodes involvement in the preauricular/parotid or submandibular/submental area. Granulomatous inflammation in other sites (axilla and upper extremity, inguinal), or older than age 10 years rarely yielded a cause. Surgical excision of granulomatous lymphadenitis has a high cure rate. Surgical excision is also more effective therapy than prolonged antibiotic oral therapy. The complication rate of children who underwent surgical excision is higher including secondary staphylococcal infection and transient or permanent facial nerve damage reason why some physicians prefer to manage deep cervical lymph nodes with antibiotics and watchful waiting.


  References:
1- Robson CD: Imaging of granulomatous lesions of the neck in children. Radiol Clin North Am. 38(5):969-77, 2000
2- Ahmed NY, Mohammed-Ali WO: A histopathological study of chronic granulomatous lymphadenitis. Saudi Med J. 28(10):1609-11, 2007
3- Harris RL, Modayil P, Adam J, Sharland M, Heath P, Planche T, Daya H: Cervicofacial nontuberculous mycobacterium lymphadenitis in children: is surgery always necessary? Int J Pediatr Otorhinolaryngol. 73(9):1297-301, 2009
4- De Corti F, Cecchetto G, Vendraminelli R, Mognato G: Fine-needle aspiration cytology in children with superficial lymphadenopathy. Pediatr Med Chir. 36(2):80-2, 2014
5- Thoon KC, Subramania K, Chong CY, Chang KT, Tee NW: Granulomatous cervicofacial lymphadenitis in children: a nine-year study in Singapore. Singapore Med J. 2014 Aug;55(8):427-31.
6- Penn EB, Goudy SL: Pediatric Inflammatory Adenopathy. Otolaryngol Clin N Am 48: 137-151, 2015

Pediatric Arterial Catheters

Indwelling arterial catheters are widely used for hemodynamic monitoring and blood sampling purposes in neonatal and pediatric patients. Arterial catheters are inserted through the umbilical artery in premature and term infants, while the radial artery route is utilized in larger children. Other alternative sites for insertion include the ulnar, brachial, axillary, dorsalis pedis and tibialis posterior arteries. Most arterial catheters in children benefit from placing them using ultrasound guidance. Almost one-third of all children with an indwelling arterial catheter will have a complication. The most common complications while using arterial catheters include catheter-related infection or inflammation, mechanical complications, embolic or thrombotic complications and bleeding. Arterial thrombosis may cause serious short-term and/or long-term complications in children, including skin necrosis, threatened limb or organ viability, leg length differences, claudication, and loss of arterial access. Another important complication of arterial catheters is bleeding when antithrombotic therapy is utilized. Possible explanations for the thrombogenicity of intraarterial catheters include damage to the vessel wall, the foreign surface, and disruption of the blood flow. This complication increases when the femoral route is utilized when compared with the radial artery. In cases where the umbilical artery is catheterized, renal flow and changes should be closely monitored. Insertion attempts of the arterial catheter at multiple sites during the admission and the presence of more than one provider participating in line placement are significant risk factors for subsequent complications. Mechanical complications or line malfunction includes leaking, removed by patient, nonfunctional, no blood return, blanching, occluded or swelling at the line site. Pronovost's prospective checklist for arterial line safety has had success in reducing the frequency of at least the most serious complications in children.


References:
1- Brotschi B, Hug MI, Latal B, Neuhaus D, Buerki C, Kroiss S, Spoerri C, Albisetti M: Incidence and predictors of indwelling arterial catheter-related thrombosis in children. J Thromb Haemost. 9(6):1157-62, 2011
2- Imamura T, Momoi N, Go H, Ogasawara K, Kanai Y, Sato M, Goto A, Hosoya M: Evaluation of arterial catheter management in very preterm neonates: peripheral artery versus umbilical artery.Fukushima J Med Sci. 58(1):1-8, 2012
3- Schindler E, Schears GJ, Hall SR, Yamamoto T: Ultrasound for vascular access in pediatric patients. Paediatr Anaesth. 22(10):1002-7, 2012
4- Molina Caballero AY, Martinez Merino, Parez Martinez et al: [Peripherally inserted central catheters: Savings, comfort, many advantages]. Cir Pediatr. 29(3):96-100, 2016
5- Hebal F, Sparks HT, Richlik KI, et al: Pediatric arterial catheters: Complications and associated risk factors. J Pediatr Surg (2017), http://dx.doi.org/10.1016/j.jpedsurg.2017.08.057



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