Psu vol 18 no 01, 2002

Vol 18 No 01 JANUARY 2002
Spontaneous Pneumothorax
Most pneumothorax in children are the result of blunt/open chest trauma, mechanicalventilation (barotrauma), bronchial asthma or an infectious pulmonary process. Primaryspontaneous pneumothorax (PSP) is rare in children with most cases seen in adolescentmales with thin body habitus. Main presenting symptoms consist of chest pain, cough andshortness of breath. Recurrence is high in this older population of children. PSP is usuallythe result of: 1) a ruptured apical bleb or bullae in three-fourth cases, 2) destructiveparenchymal disease (cystic fibrosis, AIDS), or 3) alveolar rupture due to proximal airwayobstruction. Initial management consists of oxygen supplementation for smallpneumothorax less than 15% with no tension physiology present. Chest tube drainage isneeded for medium or large size pneumothorax. Recurrence or persistent pneumothoraxis managed with video-assisted thoracoscopic surgery (VATS) by ablating with endoscopicstapling (Endo GIA), suturing or ligating using an endoloop technique the apical bullaefollowed by pleurodesis. Pleurodesis can be done chemically or surgically. Chemicalpleurodesis is achieved with such agents as talc, tetracycline, bleomycin or quinacrineinstillation. Mechanical pleurodesis carries a lower recurrent rate and can be achieved byabrasion or electrocoagulation. Most common complication is persistent air leak. VATS isa fast, cost-effective method of treatment for PSP with less morbidity. References:
1- Poenaru D, Yazbeck S, Murphy S: Primary spontaneous pneumothorax in children. J Pediatr Surg
29(9):1183-5, 1994
2- Yim AP: Video-assisted thoracoscopic suturing of apical bullae. An alternative to staple resection in the
management of primary spontaneous pneumothorax. Surg Endosc 9(9):1013-6, 1995
3- Wilcox DT, Glick PL, Karamanoukian HL, Allen JE, Azizkhan RG: Spontaneous pneumothorax: a
single-institution, 12-year experience in patients under 16 years of age. J Pediatr Surg 30(10):1452-4, 1995
4- Bertrand PC, Regnard JF, Spaggiari L, Levi JF, Magdeleinat P, Guibert L, Levasseur P: Immediate and
long-term results after surgical treatment of primary spontaneous pneumothorax by VATS. Ann Thorac Surg
61(6):1641-5, 1996
5- Cook CH, Melvin WS, Groner JI, Allen E, King DR: A cost-effective thoracoscopic treatment strategy for
pediatric spontaneous pneumothorax. Surg Endosc 13(12):1208-10, 1999
6- Chan P, Clarke P, Daniel FJ, Knight SR, Seevanayagam S: Efficacy study of video-assisted thoracoscopic
surgery pleurodesis for spontaneous pneumothorax. Ann Thorac Surg 71(2):452-4, 2001
Gastrointestinal Autonomic Nerve Tumor
Gastrointestinal Autonomic Nerve Tumor (GANT), also known as plexosarcoma, is a veryrare recently described aggressive malignant spindle cell tumor that arises from theautonomic myenteric plexus of the bowel wall. Tumor mostly originates in the small intestine and stomach with a clinical course characterize by either local or distantmetastases (liver). Histologically GANT is a low-grade, epithelioid or spindle-cellneoplasms that can be distinguished from the other gastrointestinal stromal tumors on thebasis of its unique electron microscopy ultrastructural features. Distinction of GANT fromother stromal tumors is not possible based on imaging studies. The tumor is solid andcystic, hemorrhagic, often transmural and usually involving omentum, mesentery andretroperitoneum. Signs and symptoms include abdominal pain, mass, gastrointestinalbleeding, perforation and anemia. Tumor can present with volvulus. In children they occurin females during adolescent years (mean age 12 years) mostly involving the stomach.
Primary management consists of surgical resection which is curative, leavingchemotherapy for residual or recurrent tumor. Prognosis is good for younger patients. References:
1- Segal A, Carello S, Caterina P, Papadimitriou JM, Spagnolo DV: Gastrointestinal autonomic nerve tumors:
a clinicopathological, immunohistochemical and ultrastructural study of 10 cases. Pathology 26(4):439-47,
2- Kodet R, Snajdauf J, Smelhaus V: Gastrointestinal autonomic nerve tumor: a case report with electron
microscopic and immunohistochemical analysis and review of the literature. Pediatr Pathol 14(6):1005-16,
3- Rueda O, Escribano J, Vicente JM, Garcia F, Villeta R: Gastrointestinal autonomic nerve tumors
(plexosarcomas). is A radiological diagnosis possible? Eur Radiol 8(3):458-60, 1998
4- Kerr JZ, Hicks MJ, Nuchtern JG, Saldivar V, Heim-Hall J, Shah S, Kelly DR, Cain WS, Chintagumpala MM:
Gastrointestinal autonomic nerve tumors in the pediatric population: a report of four cases and a review of the
literature. Cancer 85(1):220-30, 1999
5- Lee JR, Joshi V, Griffin JW Jr, Lasota J, Miettinen M: Gastrointestinal autonomic nerve tumor:
immunohistochemical and molecular identity with gastrointestinal stromal tumor. Am J Surg Pathol
25(8):979-87, 2001
Allgrove Syndrome
Achalasia, Alacrima & isolated Adrenal Insufficiency is also known as triple-A or Allgrovesyndrome. The high degree of consanguinity usually identified supports an autosomalrecessive mode of inheritance for this disorder. Alacrima is the earliest and mostconsistent clinical sign of Allgrove syndrome. All affected children shows esophagealdysmotility even in the absence of symptomatic dysphagia. The adrenal insufficiency isdue to adrenocorticotropic hormone (ACTH) resistance. Other associated neurologicalabnormalities include autonomic, sensory, and upper- and lower-motor neuropathy,deafness, and mental retardation. Genetically the syndrome has been mapped tomutations in chromosome 12q13 region near the type II keratin gene cluster. Though mostcases arise in children between the ages of two and eight years, a few cases originateduring early adult years. Management for alacrima consists of artificial tears replacement.
Adrenal insufficiency will need steroid replacement. Achalasia is treated with a modifiedHeller esophagomyotomy which can be done open or laparoscopically. A fewneurologically impaired children will need gastrotomy feedings.
1- Moore PS, Couch RM, Perry YS, Shuckett EP, Winter JS: Allgrove syndrome: an autosomal recessive
syndrome of ACTH insensitivity, achalasia and alacrima. Clin Endocrinol (Oxf) 34(2):107-14, 19912- Stratakis CA, Lin JP, Pras E, Rennert OM, Bourdony CJ, Chan WY: Segregation of Allgrove (triple-A)syndrome in Puerto Rican kindreds with chromosome 12 (12q13) polymorphic markers. Proc Assoc AmPhysicians 109(5):478-82, 19973- Huebner A, Yoon SJ, Ozkinay F, Hilscher C, Lee H, Clark AJ, Handschug K: Triple A syndrome--clinicalaspects and molecular genetics. Endocr Res 26(4):751-9, 20004- Sandrini F, Farmakidis C, Kirschner LS, Wu SM, Tullio-Pelet A, Lyonnet S, Metzger DL, Bourdony CJ,Tiosano D, Chan WY, Stratakis CA: Spectrum of Mutations of the AAAS Gene in Allgrove Syndrome: Lack ofMutations in Six Kindreds with Isolated Resistance to Corticotropin. J Clin Endocrinol Metab 86(11):5433-7,2001 * Edited by: Humberto L. Lugo-Vicente, MD, FACS, FAAP
Professor/Associate Director of Pediatric Surgery, University of Puerto Rico School of Medicine and University Pediatric Hospital, Rio Piedras, Puerto Rico.
Address: P.O. Box 10426, Caparra Heights Station, San Juan, Puerto Rico USA 00922-0426. Tel (787)-786-3495 Fax (787)-720-6103 E-mail: [email protected]


Corresponding author available concerning management of patients who have Samuel J. Mann, MD New York Presbyterian Hospital-Weill/Cornell Medical School, a pheochromocytoma, fewer than two dozen papers deal 450 East 69th Street, New York, NY 10021, USA. with the 98% who do not. Doctors and researchers simply do not know how to manage these patients. Current Hypertension Reports 2008, 1

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