PEDIATRIC SURGERY UPDATE ©
VOLUME 07, 1996


VOL 07 NO 01 JULY 1996

Fibrosarcoma

Fibrosarcoma, a malignant tumor of fibrous tissue, is a unusual neoplasms in infants and children. It comprises 10% of all soft-tissue sarcomas in children and the most common during the neonatal period. Anaplastic spindle-shaped cells arranged in a herringbone pattern with abnormal mitosis, nuclear pleomorphism and increase basophilia are characteristic pathological findings. Two peaks in the incidence are affected: infants and young children under the age of five (congenital variety) and patients between 10 and 15 years of age. A slight male predominance is reported. Clinically the child presents with a rapidly enlarging mass or swelling in the soft tissue. Sites of origin in order of decreasing frequency are: the extremity, head and neck, trunk, and retroperitoneum. Most important prognostic factors are: site of origin, and extent of disease at diagnosis. Fibrosarcoma is a locally aggressive tumor. Tumors in infants have a more benign course. Extremity lesions have a low rate of recurrence and better prognosis. Axial tumors have a high rate of metastasis and death from disease. Adequate biopsy is necessary for diagnosis. Mainstay of therapy consists of surgical resection by wide local excision or amputation for neurovascular bundle involvement or development of a dysfunctional extremity. Chemotherapy (VAC) is a useful adjunct for unresectable metastatic or primary disease and to alter the surgical approach to bulky tumors. Overall survival is 80% at five years.

References
1-Baker S, Koenig J, Ternberg J: Congenital Fibrosarcoma. J Pediatr Surg 22(7): 665-670, 1987
2- Dillon P, Whalen T, Azizkhan R: Neonatal Soft Tissue Sarcomas: The Influence of Pathology in Treatment and Survival. J Pediatr Surg 30: 1038-1041, 1995
3- Grier H, Perez A, Weinstein H: Chemotherapy for Inoperable Infantile Fibrosarcoma.  Cancer 56: 1507-1510, 1985
4- Phillip Lanzkowski: Pediatric Oncology, McGraw-Hill, Inc. 1983, pp. 279-282.
5- Nerfell JP, Berg JW, Godwin D, et al: A Retrospective Epidemiologic Study of Pediatric  Fibrosarcoma. J Pediatr Surg 13: 735-739, 1978
6- Pizzo PA: Principles and Practice of Pediatric Oncology, JB Lippincott Co, 1989, pp.679-682.

7- Soule E, Pritchard D: Fibrosarcoma in Infants and Children. Cancer 40: 1711-1720, 1977

Congenital Adrenal Hyperplasia

Congenital adrenal hyperplasia (CAH) involves a functional defect in any of the five enzymatic steps required for cortisol synthesis, most commonly 21- (involved in 90-95% of cases) and 11-hydroxylase level. This primary genetic defect transmitted as autosomal recessive impairs the ability of the adrenal cortex to synthesize cortisol causing increase feedback secretion of ACTH and adreno-cortical hyperplasia of the gland. Increase output of steroids proximal to the block (androgenic precursors) causes virilization in affected males and females. Its more severe form is associated with aldosterone deficiency and life-threatening salt wasting. Female pseudohermaphrodite due to virilizing CAH is the most frequent form of intersexuality found. The phenotypic picture varies from mild clitoral enlargement alone to complete masculinization of the urethral meatus at the tip of the penis. Prenatal diagnosis (southern blotting of DNA) is based on finding the disease gene on the short arm of chromosome 6. Likewise management in the mother (dexamethasone) is started empirically until the affected status is known by chorionic villus sampling. After birth management consists of cut-back or flap vaginoplasty with clitoral recession at 3-6 months of age. Children with high vaginal entry proximal to the urethra external sphincter can undergo early one-stage reconstruction at 8-12 months of age. Long term surgical results of female children show adequate sexual identification, reproduction, intellectual functioning and acceptable genitalia.

References
1- New MI: Genetic Disorders of Adrenal Hormone Synthesis. Horm Res 37:22-33, 1992
2- Donahue PK, Gustafson M: Early One Stage Surgical Reconstruction of Extremely High Vagina in Patients with Congenital Adrenal Hyperplasia. J Pediatr Surg 29(2): 352-358, 1994
3- Coran AG, Polley JR: Surgical Management of Ambiguous Genitalia in the Infant and Child. J Pediatr Surg 26(7): 812-820, 1991
4- Bhatia V, Shukla R, Mishra SK, et al: Adrenal Tumor Complicating Untreated 21-hydroxylase Deficiency in a 5.5 year old boy: Arch Pediatr Adoles Med 147: 1321-1323, 1993
5- Srikanth MS, West BR, Ishitani M, et al: Benign Testicular Tumors in Children with Congenital Adrenal Hyperplasia. J Pediatr Surg 27(5): 634-641, 1992
6- Hurtig AL, Radhakrishnan J, Reyes, HM, et al: Psychological Evaluation of Treated Females with Virilizing Congenital Adrenal Hyperplasia. J Pediatr Surg 18(6): 887-893, 1983
7- Vanderkamp JJ, Slijper ME, Brandenburg JJ, et al: Evaluation of Young Women with Congenital Adrenal Hyperplasia. Horm Res 37: 45-49, 1992

8- Siebemann RE: Disorder of Steroid Hormone Biosynthesis. Progress Pediatr Surg 16: 171-183, 1983

Microgastria

Microgastria is a rare congenital anomaly of arrested foregut development resulting in a small stomach with minimal reservoir capacity. Frequently associated to: malrotation, asplenia, cardio-pulmonary, and limb anomalies. Clinically the infant develops feeding intolerance: postprandial vomiting, gastro-esophageal reflux, aspiration pneumonia, and failure to thrive. Diagnosis is made by UGIS showing a small saccular or tubular midline stomach, proximally dilated esophagus, and incompetent cardia. Management consists of small frequent feeding in less severe cases to creation of a double lumen Roux-en-Y jejunal pouch (Hunt-Lawrence Pouch) for food storage in symptomatic children. This procedure (see figure) provides a pouch for food intake, improves nutritional status, ease drainage and prevents alkaline reflux esophagitis. Vitamin's B-12 supplement is needed due to decrease production of Intrinsic factor.

References
1- Velasco AL, Holcomb GW, Templeton JM, et al: Management of Congenital Microgastria. J Pediatr Surg 25(2): 192-197, 1990
2- Hasegawa S, Kohno S, Tamura K, et al: Congenital Microgastria in an Infant with the VACTERL Association. J Pediatr Surg 28(6): 782-784, 1993
3- Hoehner JC, Kimura K, Soper RT: Congenital Microgastria. J Pediatr Surg 29(12): 1591-1593, 1994
4- Moulton SL, Bouvet M, Lynch FP: Congenital Microgastria in a Premature Infant. J Pediatr Surg 29(12): 1594-1595, 1994

5- Cunniff C, Olney AH: Congenital Microgastria and Limb reduction Defects. Pediatrics 91(6): 1192-1195, 1993


VOL 07 NO 02 AUGUST 1996

Meconium Ileus-like Syndrome

Meconium-associated intestinal obstruction during the neonatal period can be the result of an intrauterine perforation (meconium peritonitis), a distal colonic plug (meconium plug syndrome) or intraluminal tenacious meconium associated to decreased pancreatic enzyme (trypsin) activity from cystic fibrosis (meconium ileus). Although most cases of meconium ileus (MI) are associated to mucoviscidosis, 10-20% are sweat-test and genetic marker negative. This is what we call meconium ileus-like syndrome. Most babies with MI-like syndrome are markedly premature (22 to 30 weeks) with very low birth weight. Postnatal factors associated are patent ductus arteriosus, hyaline membrane disease, and intraventricular hemorrhage. The syndrome is the result of a combination of intestinal hypoperfusion and dysmotility due to hemodynamic abnormalities and immaturity of the myenteric plexus of the ileum and colon respectively. Clinically, they have absent stooling during the first two weeks of life, diffuse abdominal distension, retrograde peristalsis, meconium with a high content of albumin, obstruction in the distal ileum, and microcolon. Medical management consists of a gastrograffin enema (is diagnostic and therapeutic), oral gastrograffin, and 10% acetylcysteine (mucomyst) as enteral cleansing agent. Surgery is indicated for progressive clinical deterioration or development of pneumoperitoneum. Follow-up shows no gastrointestinal or pulmonary dysfunction.
 
References
1- Greenholz SK, Perez C, Wesley JR, et al: Meconium Obstruction in the Markedly Premature Infant. J Pediatr Surg 31(1): 117-120, 1996
2- Chang PY, Huang FY, Yeh ML, et al: Meconium Ileus-Like Condition in Chinese Neonates. J Pediatr Surg 27(9): 1217-1219, 1992
3- Del Pin CA, Czyrko C, Ziegler MM, et al: Management and Survival of Meconium Ileus. Ann Surg 215(2); 179-185, 1992
4- Tillig E, Robel R, Vogtmann C, et al: Severe protracted intrauterine impaired perfusion--a cause of enteral motility disorder in the premature infant] Z Geburtshilfe Neonatol 199(5):190-4, 1995
5- Toyosaka A, Tomimoto Y, Nose K, et al: Immaturity of the myenteric plexus is the aetiology of meconium ileus without mucoviscidosis: a histopathologic study. Clin Auton Res 4(4):175-84, 1994
6- Fakhoury K, Durie PR, Levison H, et al: Meconium ileus in the absence of cystic fibrosis. Arch Dis Child 67(10 Spec No):1204-6, 1992
7- Bregante Ucedo JI, Hervas Palazon JA, Henales Villate V, et al: Meconial peritonitis without mucoviscidosis. Report of tree cases An Esp Pediatr 33(3):289-92, 1990
8- Amodio J, Berdon W, Abramson S, et al: Microcolon of prematurity: a form of functional obstruction. AJR Am J Roentgenol 146(2):239-44, 1986

9- Shigemoto H, Endo S, Isomoto T, et al: Neonatal meconium obstruction in the ileum without mucoviscidosis. J Pediatr Surg 13(6):475-9, 1978

Meckel's Diverticulum

Meckel's diverticulum (MD), the pathologic structure resulting from persistence of the embryonic vitelline duct (yolk stalk), is the most prevalent congenital anomaly of the GI tract. MD can be the cause of: gastrointestinal bleeding (most common complication), obstruction, inflammation and umbilical discharge in children and 50% occur within the first two years of life. Diagnosis depends on clinical presentation. Rectal bleeding from MD is painless, minimal, recurrent, and can be identified using 99mTc- pertechnetate scan; contrasts studies are unreliable. Persistent bleeding requires arteriography or laparotomy if the scan is negative. Obstruction secondary to intussusception, herniation or volvulus presents with findings of fulminant, acute small bowel obstruction, is diagnosed by clinical findings and contrast enema studies. The MD is seldom diagnosed preop. Diverticulitis or perforation is clinically indistinguishable from appendicitis. Mucosal polyps or fecal umbilical discharge can be caused by MD. Overall, complications of Meckel's are managed by simple diverticulectomy or resection with anastomosis. Laparoscopy can confirm the diagnosis and allow resection of symptomatic cases. Removal of asymptomatic Meckel's identified incidentally should be considered if upon palpation there is questionable heterotopic (gastric or pancreatic) mucosa (thick and firm consistency) present.
 
References
1- Panuel M, Campan N, Delarue A, et al: Ultrasonographic diagnosis and laparoscopic surgical treatment of Meckel's diverticulum. Eur J Pediatr Surg 4(6):344-5, 1994
2- Moore TC: Omphalomesenteric Duct Malformation. Semm Pediatr Surg 5(2): 116-123, 1996
3- Mackey WC, Dineen P: A Fifty Year Experience with Meckel's Diverticulum. 156: 56-64, 1983
4- Benson CD: Surgical Implications of meckel's Diverticulum. In Ravitch's Textbook of Pediatric Surgery, Year Book Medical Publishers, 1979, pag 955-964
5- Amoury RA: Meckel's Diverticulum. In Welch's Textbook of Pediatric Surgery, Year Book Medical Publishers, 1986, pag 859-867

6- Scharli AF: Vitello-intestinal disorders. In Freeman's et al Surgery of the Newborn. Churchill Livingstone, 1994, pag 243-250

Nesidioblastosis

Persistence of early fetal cells leading to neonatal hyperinsulinism characterized by severe hypoglycemia can be caused by Nesidioblastosis, one of the islet cell dysmaturation syndrome. Diagnosis rests on four criteria: the presence of increased insulin levels during hypoglycemia, low urinary excretion of ketone bodies, the need to infuse more than 15/mg/kg/min glucose to maintain normal serum levels, and a positive response to glucagon. Seizures, cyanosis, and central respiratory disturbances are the presenting symptoms of hypoglycemia in most cases, and prompt diagnosis avoids the sequelae of mental retardation. Subtotal (95%) pancreatectomy should be done early in these patients if hypoglycemia cannot be controlled with medical therapy (diazoxide) or side effects of therapy are documented. Pancreatic function is not seriously impaired in most patients after subtotal pancreatectomy. Avoiding the need of pancreatectomy in selected patients by long-term use of octreotide (somatostatin analog) may be possible.

References
1-Samoud A, Dey D, Brauner R, Doagi M; Kallel H, Boubaker S, Osman R, Ben Dridi MF:  [Nesidioblastosis. Apropos of 12 new cases] Ann Pediatr (Paris) 37(10):651-5, 1990
2- Gottschalk E, Luders H, Scheerschmidt G, Hauch A:  [Experiences with nesidioblastosis]  Zentralbl Chir 115(22):1441-7, 1990
3- Glaser B, Phillip M, Carmi R, Lieberman E, Landau H: Persistent hyperinsulinemic hypoglycemia of infancy (nesidioblastosis): autosomal recessive inheritance in 7 pedigrees. Am J Med Genet 37(4):511-5, 1990
4- Bjerke HS, Kelly RE Jr, Geffner ME, Fonkalsrud EW: Surgical management of islet cell dysmaturation syndrome in young children. Surg Gynecol Obstet 171(4):321-5, 1990
5-.Dunger DB, Burns C, Ghale GK, Muller DP, Spitz L, Grant DB: Pancreatic exocrine and endocrine function after subtotal pancreatectomy for nesidioblastosis J Pediatr Surg 23(2):112-5, 1988
6- Low LC, Yu EC, Chow OK, Yeung CY, Young RT: Hyperinsulinism in infancy. Aust Paediatr J 25(3):174-7, 1989
7- Warden MJ,  German JC, Buckingham BA: The surgical management of hyperinsulinism in infancy due to nesidioblastosis. J Pediatr Surg 23(5):462-5, 1988
8- Sempoux C, Poggi F, Brunelle F, Saudubray JM, Fekete C, Rahier J: Nesidioblastosis and persistent neonatal hyperinsulinism. Diabete Metab 21(6):402-7, 1995
9- Tauber MT, Harris AG, Rochiccioli P: Clinical use of the long acting somatostatin analogue octreotide in pediatrics.  Eur J Pediatr 153(5):304-10, 1994
10- Glaser B, Hirsch HJ, Landau H: Persistent hyperinsulinemic hypoglycemia of infancy: long-term octreotide treatment without pancreatectomy [see comments]  J Pediatr 123(4):644-50, 1993
12- Willberg B, Muller E: Surgery for Nesidioblastosis. Progress Pediatr Surg 26: 76-83, 1991
13- Dobroschke J, Linder R, Otten A: Surgical Treatment of Nesidioblastosis in Childhood. Progress Pediatr Surg 26: 84-91, 1991

14- Dohrmann P, Mengel W, Splieth J: Total pancreatectomy in a Case of Nesidioblastosis Due to Persisting Hyperinsulinism Following Subtotal Pancreatectomy. Progress Pediatr Surg 26: 92-95, 1991


VOL 07 NO 03 SEPTEMBER 1996

MMIHS

First described by Berdon in 1976, the Megacystis, Microcolon Intestinal Hypoperistalsis Syndrome (MMIHS), represents a rare lethal form of neonatal intestinal obstruction. It is seen in newborn girls showing complete intestinal obstruction (absent meconium output and bilious vomiting), large bladder causing a distended abdomen (dilated urinary and upper gastrointestinal tracts without distal anatomic obstruction), microcolon, absent peristalstic activity, lax abdominal musculature, and malrotation. Positive family history suggests autosomal recessive inheritance. Usual diagnostic studies display: KUB (lacking gas shadow with ground glass appearance), barium enema (malrotated, unused microcolon), cystogram (dilated bladder without distal obstruction), IVP (hydroureter and hydronephrosis), UGIS (foreshortened midgut), and suction rectal biopsy (ganglion cell present). Surgical and postmortem specimens' describe thin longitudinal muscle coats with vacuolation and degeneration of smooth muscle cells of bowel and bladder, increase connective tissue between them, and abundant mature ganglion cells (referred as hollow visceral myopathy). Initial management is gastrointestinal decompression, and parenteral nutrition (TPN). MMIHS is not a surgical remediable condition. The outcome is generally fatal.
 
References
1- Leibowitz S, Bodian M: A Study of the Vesical ganglia in children and the relationship to the megaureter megacystis syndrome and Hirschsprung's Disease. J Clin Path 16:342, 1963
2- Amoury RA, Fellows RA, Goodwin CD, et al: Megacystis-Microcolon-Intestinal Hypoperistalsis Syndrome: A Cause of Intestinal Obstruction in the Newborn Period. J Pediatr Surg 12(6): 1063, 1977
3- Kirtane J, Talwalker V, Dastur DK: Megacystis, Microcolon, Intestinal Hypoperistalsis Syndrome: Possible Pathogenesis. J Pediatr Surg 19(2): 206, 1984
4- Bagwell CE, Filler RM, Cutz E, et al: Neonatal Intestinal Pseudoobstruction. J Pediatr Surg 19(6): 732, 1984
5- Puri P, Tsoji M: Megacystis-microcolom-intestinal hypoperistalsis syndrome (neonatal hollow visceral myopathy). Pediatr Surg Int 7:18, 1992

6- Goldberg M, Pruchniewski D, Beale PG, et al: Megacystis-microcolon-intestinal hypoperistalsis syndrome. Pediatr Surg Int 11:246, 1996

Intestinal Atresias

Intestinal atresias are the product of a late intrauterine mesenteric vascular accident as attested by Louw and Barnard in 1955. They are equally distributed from the ligament of treitz to the ileocecal junction. There is proximal bowel dilatation, with distal (unused) micro-bowel. The diagnosis is suspected with maternal history of polyhydramnios (the higher the atresia), bilious vomiting, abdominal distension and obstipation. KUB shows "thumb-size" dilated bowel loops, and barium enema a microcolon of disuse. Louw classified them into: Type I: an intraluminal diaphragm with seromuscular continuity. Type II: cord-like segment between the bowel blinds ends. Type IIIA: atresia with complete separation of blind ends and V-shaped mesenteric defect (see figure), the most commonly found. Type IIIB: jejunal atresia with extensive mesenteric defect and distal ileum acquiring its blood supply entirely from a single ileocolic artery. The distal bowel coils itself around the vessel, giving the appearance of an "apple peel"deformity. Type IV: multiple atresias of the small intestine. After preoperative stabilization, treatment consists of exploratory laparotomy, resection of proximal dilated intestine, and end to oblique anastomosis in distal jejuno-ileal atresias. Tapering jejunoplasty with anastomosis is preferred in proximal defects.
 
References
1-de Lorimier AA;  Harrison MR: Intestinal plication in the treatment of atresia. J Pediatr Surg 18(6):734-7, 1983
2-Dickson JA: Apple peel small bowel: an uncommon variant of duodenal and jejunal atresia. J Pediatr Surg 5(6):595-600, 1970
3-Teja K;  Schnatterly P;  Shaw A: Multiple intestinal atresias: pathology and pathogenesis. J Pediatr Surg 16(2):194-9, 1981
4-Miller RC:  Complicated intestinal atresias. Ann Surg 189(5):607-11, 1979

5-DeLorimier AA;  Fonkalsrud EW;  Hays DM: Congenital atresia and stenosis of the jejunum and ileum. Surgery 65(5):819-27, 1969

OK-432

In 1986, Ogita, et al. observed that intracystic injection of OK432 (lyophilized product of Streptococcus pyogenes) caused cystic (hygromas) lymphangiomas to become inflamed and led to subsequent cure of the lesion without side effects. The number of patients treated with OK432 now stands at more than 70. Results of a cooperative study for OK432 therapy in Japan were excellent. OK432 caused inflammatory reaction, but did not damage the overlaying skin or lead to scar formation. Total or near-total shrinkage of the lesions, without serious complications, was noted in more than 90% of cystic type lymphangiomas (1 to 7 injections; mean, 1.8). On the other hand, this was noted in 50% of cavernous type lymphangiomas (1 to 18 injections; mean, 6.3). Side effects occurring during treatment were: fever, swelling and reddening of the tumor, increased white cell count, and increased CRP level. None of these reactions were serious. Swelling of the lymphangioma was usually insignificant. Depending on location, this could be a risk. No anaphylactic or other reactions to treatment were observed. In patients who were cured, there has been no recurrence throughout the followup period (6 months to 9 years after the last injection of OK432). This segment was written by: Shuhei Ogita, MD, Division of Surgery, Children's Research Hospital, Kyoto Pref Univ of Med, Kyoto 602, Japan, e-mail: s-ogita@koto.kpu-m.ac.jp URL: http://www.asahi-net.or.jp/~VF6S-OGT/index.html

References
1- Ogita S, Tsuto T, Nakamura K, Deguchi E, Tokiwa K, Iwai N: OK-432 therapy for lymphangioma in children: why and how does it work? J Pediatr Surg 31(4):477-80, 1996
2- Ogita S, Tsuto T, Nakamura K, Deguchi E, Iwai N: OK-432 therapy in 64 patients with lymphangioma. J Pediatr Surg 29(6):784-5, 1994
3- Ogita S, Tsuto T, Deguchi E, Tokiwa K, Nagashima M,  Iwai N: OK-432 therapy for unresectable lymphangiomas in children. J Pediatr Surg 26(3):263-8; discussion 268-70, 1991

4- Ogita S, Tsuto T, Tokiwa K, Takahashi T: Intracystic injection of OK-432: a new sclerosing therapy for cystic hygroma in children. Br J Surg 74(8):690-1, 1987


VOL 07 NO 04 OCTOBER 1996

Epignathus

The term epignathus describes a rare benign teratoma (poorly organized tissues derived from elements of the three embryonic germ cell layers) that originates from the oropharynx associated with serious airway compromise upon birth. The tumor grows from the palate, mandible, or base of the skull and protrudes through the mouth. The pedunculated epignathus consist of structure confined to the head and neck attached either to nasopharynx in the region of the basisphenoid or the dorsal aspect of palate and pterygoid plates (Rathke's pouch). Occurs predominantly in females, and clinical picture includes: dyspnea, suffocation, difficulty in sucking, swallowing and vomiting. In the past, mortality from obstruction to respiration upon birth was very high. Recent advances in prenatal diagnosis permits the perinatal multidisciplinary team (neonatologist, anesthesiologist and pediatric surgeon) to take advantage of the neonatal airway before cord clamping by either endotracheal intubation or if necessary tracheostomy after delivery by cesarean section. Debulking surgical resection removing as much tumor as possible and avoiding speech or deglutition morbidity ensues for large masses. Small epignathi are easily removed. Prognosis depend on histology and associated deformity. Follow-up for recurrence is warranted using imaging studies and alpha-fetoprotein serum levels. Chemotherapy may be reserved for unresectable recurrent tumors.
 
References
1- Levine AB, Alvarez M, Wedgwood J, Berkowitz RL, Holzman I: Contemporary management of a potentially lethal fetal anomaly: a successful perinatal approach to epignathus. Obstet Gynecol 76(5 Pt 2):962-6, 1990
2- Todd DW, Votava HJ, Telander RL, Shoemaker CT: Giant epignathus. A case report. Minn Med 74(7):27-8, 1991
3- Maeda K, Yamamoto T, Yoshimura H, Itoh H: Epignathus: a report of two neonatal cases. J Pediatr Surg 24(4):395-7, 1989
4- Smart PJ, Schwarz C, Kelsey A: Ultrasonographic and biochemical abnormalities associated with the prenatal diagnosis of epignathus. Prenat Diagn 10(5):327-32, 1990
5-  Senyuz OF, Rizalar R, Celayir S, Oz F: Fetus in fetu or giant epignathus protruding from the mouth. J Pediatr Surg 27(12):1493-5, 1992
6- Zakaria MA: Epignathus (congenital teratoma of the hard palate): a case report. Br J Oral Maxillofac Surg 24(4):272-6, 1986
7- Hatzihaberis F, Stamatis D, Staurinos D: Giant epignathus. J Pediatr Surg 13(6):517-8, 1978
8- Catalano PJ, Urken ML, Alvarez M, Norton K, Wedgewood J, Holzman I, Biller HF: New approach to the management of airway obstruction in "high risk" neonates. Arch Otolaryngol Head Neck Surg 118(3):306-9, 1992

9- Valente A, Grant C, Orr JD, et al: Neonatal Tonsillar Teratoma. J Pediatr Surg 23(4): 364-366, 1988

Pure TEF

Congenital isolated tracheo-esophageal fistula (TEF) occurs as 4-6% of the disorders of the esophagus bringing problems during early diagnosis and management. More than H-type is N-type, due to the obliquity of the fistula from trachea (carina or main bronchi) to esophageal side (see the figure) anatomically at the level of the neck root (C7-T1). Pressure changes between both structure can cause entrance of air into the esophagus, or esophageal content into the trachea. Thus, the clinical manifestation that we must be aware for early diagnosis are: cyanosis, coughing and choking with feedings, recurrent chest infections, persistent gastrointestinal distension with air, and hypersalivation. Diagnosis is confirmed with a well-done esophagogram, or video-esophagogram (high success rates, establish level of the TEF). Barium in the trachea could be caused by aspiration during the procedure. Upon radiologic doubt bronchoscopy should be the next diagnostic step. Any delay in surgery is generally due to delay in diagnosis rather than delay in presentation. Management consists of surgical closure of the TEF through a right cervical approach. Hint: a small guide-wire threaded through the fistula during bronchoscopy may be of some help. Working in the tracheo-esophageal groove can cause injury to the recurrent laryngeal nerve with vocal cord paralysis. Recurrence after closure is rare.

References
1- Beasley SW, Myers NA, Auldist AW: Oesophageal Atresia Chapter 13 Trachea-oesophageal fistula: the H' fistula, Chapman and Hall Medical , 1991 pag 193-207
2- Kirk JM; Dicks-Mireaux C: Difficulties in diagnosis of congenital H-type trachea-oesophageal fistulae. Clin Radiol 40(2):150-3, 1989
3- Yazbeck S, Dubuc M: [Congenital tracheoesophageal fistulas without esophageal atresia]Chir Pediatr 24(2):113-6, 1983
4- Geiss S, Clavert JM, Bientz J, Nord M, Sauvage P, Benoit M, Dissel B, Beauvais P: [Isolated tracheoesophageal fistulas. Apropos of 3 cases revealed in the newborn] Chir Pediatr 29(4):205-7, 1988
5- Spitz L, Hitchock RJ: Oesophageal atresia and tracheo-oesophageal fistula In Freeman's et al Surgery of the Newborn. Churchill Livingstone, 1994, pag 368-369

6- Holder TM: Esophageal Atresia and Tracheoesophageal Fistula In Ashcraft & Holder Pediatric Esophageal Surgery. Grune & Stratton, 1986, pag 53-71

Internet

Internet represents thousand of non-centralized computers networks worldwide connected in nodes using a public-domain standard transmission control (TCP) and (IP) internet protocol. Originally developed to provide communication after a nuclear holocaust, it gradually developed into an explosive resourceful area for: chatting, e-mailing, newsgroups, long-distance computing, and transfer of files. Then came the World Wide Web (WWW) developed in Geneva by CERN to transmit web pages through the Net using a hyper text transmission protocol (HTTP). Information as text, images, pictures, sound, music, voice, animation and video is distributed and retrievable using this protocol. Linking between web pages was possible. WWW is the fastest growing internet resource since web pages add entertainment, information, and advertisement. Nodes were divided either geographically or into institutional gateways (gov, mil, edu, com, org, and net). Future of the Net will develop into a bigger and faster multimedia circus that will change our attitude toward clinical care, investigation and publication.

References
1- http://dragonfire.net/~Flux/ihistory.html
2- http://www.forthnet.gr/forthnet/isoc/short.history.of.internet
3-gopher://gopher.isoc.org:70/00/internet/history/how.internet.came.to.be
4- De Fiore L: [Internet and medicine (editorial)] Recenti Prog Med 86(10):406-8, 1995
5- Di Giorgio CJ; Richert CA; Klatt E; Becich MJ:  E-mail, the Internet, and information access technology in pathology. Semin Diagn Pathol 11(4):294-304, 1994
6- Frisse ME; Kelly EA; Metcalfe ES:  An Internet primer: resources and responsibilities. Acad Med 69(1):20-4, 1994
7- Spooner SA: On-line resources for pediatricians. Arch Pediatr Adolesc Med 149(10):1160-8, 1995
9- Hardin, Jr., WD: The Philosophy Behind the Website. http://pedsurg.surgery.uab.edu/editorial/webed1.htm

10- Lugo-Vicente HL: The Role of Internet in Modern Medicine. Boletin AMPR 87 (4-5-6): 82-87, 1997
 

VOL 07 NO 05 NOVEMBER 1996

Rhabdomyosarcoma

Rhabdomyosarcoma (RMS) the most common soft tissue sarcoma in infants and children represents about 5-15% of all malignant solid lesions. It has a peak incidence before the age of five years, and a second surge during early adolescence. Head, neck and pelvic malignancies are more prevalent in infancy and early childhood, while trunk, extremity and paratesticular RMS is largely a disease of adolescents. RMS arises from a primitive cell type and occurs in mesenchymal tissue at almost any body site (excluding brain & bone). Predominant histologic type in infants and small children is embryonal. Botryoid RMS is a subtype of the embryonal variety, which ordinarily extends into body cavities such as bladder, nasopharynx, vagina, or bile duct. The alveolar cell type, named for a superficial similarity to the pulmonary alveoli, is the most common form found on the muscle masses of the trunk and extremities, and is seen more frequently in older children. Clinical findings, diagnostic evaluation and therapy depend upon location of the primary tumor and is beyond the scope of this review. Head and neck RMS are most common and occur in the orbit, nasopharynx, paranasal sinuses, cheek, neck, middle ear, and larynx. Most are treated by simple biopsy followed by combined therapy or preop chemotherapy and radiation followed by conservative resection. Operations for extremity lesions include wide local excision to remove as much of gross tumor as possible. The trend in management is more chemotherapy with conservative surgical therapy. Survival depends on primary site, stage of disease, and treatment given.
 
References
1- Hays DM: Rhabdomyosarcoma, In DM Hays "Pediatric Surgical Oncology" Grune & Stratton, Inc, 1986 pag 87-122
2- Chapter 12: Soft Tissue Tumors. In Isaacs Hart's Tumors of the Newborn and Infant. Mosby year Book, 1991, pag 200-208

3- Maurer HM, Ruymann FB, Pochedly C: Rhabdomyosarcoma and Related Tumors in Children and Adolescent. CRC Press, 1991

Hepatic Cysts

Hepatic cysts (HC) can be either parasitic (echinococcal) following infestation in endemic regions, acquired (after trauma or inflammatory processes), or nonparasitic (congenital) in nature. Congenital nonparasitic HC are uncommon, solitary, benign lesions that arise from aberrant development of intrahepatic biliary radicals after ischemic thrombo-embolic phenomena (vascular disruption theory). The cyst is lined with cuboidal or squamous epithelium, and there is a female and white children predominance. Although generally asymptomatic, children may manifest increased abdominal girth, vague abdominal discomfort, infection, or obstructive jaundice. Ultrasound and CT-Scan are diagnostic tools. Management may consist of: simple unroofing, complete removal by enucleation or hepatic lobectomy, internal roux-en-Y drainage, or percutaneous aspiration and sclerosis (alcohol, minocycline). The surgical alternative to use will depend on size, location (central, peripheral or dumbbell), and presence of communication with biliary system of the cyst (see figure). Some cases diagnosed prenatally or during the neonatal period have undergone slow spontaneous regression.
 
References
1- Avni EF, Rypens F, Donner C, et al: Hepatic Cysts and Hyperechogenicities: Perinatal Assesment and Unifying Theory on their Origin. Pediatr Radiol 24: 569-572, 1994
2- Nelson J, Davidson D, McKittrick JE: Simple Surgical Treatment of Nonparasitic Hepatic Cysts.  Amer Surg 58:755-756, 1992
3- Ramesh J, Walrond ER, Prussia DM, et al: Congenital Solitary Non-parasitic Cyst of the Liver.  W I Med J 44:36-37, 1995
4- Haginawa H, Kasahara A, Hayashi N, et al: Succesful treatment of a Hepatic Cyst by One-Shot Instillation of Minocycline Chloride. Gastroenterology 103: 675-677, 1992
5- Simonetti G, Profili S, Sergiacomi Glet al: Percutaneous treatment of hepatic cysts by aspiration and sclerotherapy. Cardiovasc Intervent Radiol 16(2):81-4, 1993

6- Rowe: Esentials in Pediatric Surgery Chapter 70 Hepatocellular Disorders, pag 619-624

Henoch-Schönlein Purpura

Henoch-Schönlein Purpura (HSP) is a generalized autoimmune vasculitis characterized by non-thrombocytopenic purpuric rash, arthritis, glomerulonephritis, and gastrointestinal symptoms thought to occur as diffuse IgA hypersensitivity response. Main clinical features are: age between 3 and 7 years, skin rashes followed by GI symptoms, joint symptoms, soft tissue edema, and renal involvement. GI symptoms (pain, vomiting and bleeding) occurring in almost 2/3 of cases may need prompt surgical evaluation during crisis. Some complication leading to surgical therapy includes intussusception (most common, involves small bowel alone), perforation, infarction, and massive GI bleeding. Ultrasound can show the thickened hemorrhagic infiltration of the intestinal wall, follow evolution of these lesions and identify potential surgical complications Obstructed children need sequential Ba enema and UGIS. Steroids can mask symptoms of fistula and abscess formation. Suspicion and early diagnosis of HSP after clinical, imaging and lab findings avoid unnecessary operations.
 
References
1- Katz S, Borst M, Seekri I, Grosfeld JL: Surgical evaluation of Henoch-Schonlein purpura. Experience with 110 children Arch Surg 126(7):849-53, 1991; discussion 853-4
2- Martinez-Frontanilla LA, Haase GM, Ernster JA, Bailey WC: Surgical complications in Henoch-Schonlein Purpura. J Pediatr Surg 19(4):434-6, 1984
3- Mir E:  Surgical complications in Henoch-Schonlein Purpura in childhood. Z Kinderchir 43(6):391-3, 1988
4- Wang YJ, Chi CS, Shian WJ:  Clinical studies of Henoch-Schonlein purpura in Chinese children. Chung Hua I Hsueh Tsa Chih (Taipei) 51(5):345-9, 1993
5- Couture A, Veyrac C, Baud C, Galifer RB, Armelin I: Evaluation of abdominal pain in Henoch-Schonlein syndrome by high frequency ultrasound [see comments]Pediatr Radiol 22(1):12-7, 1992
6- Cull DL, Rosario V, Lally KP, et al: Surgical Implications of Henoch-Schönlein Purpura. J Pediatr Surg 25(7): 741-743, 1990

7- van den Broek RW, van Rossum MA, van Duinen CM: A new surgical complication related to corticosteroids in a patient with Henoch-Schonlein purpura.J Pediatr Surg 30(9):1341-3, 1995


VOL 07 NO 06 DECEMBER 1996

Internet Part 2

The Internet consists of thousands of interconnected networks. It works by way of local and long-drag connections, routers, servers, protocols and browsers. LOCAL: Using regular telephone lines and a modem your personal computers connect to the internet service provider (ISP) or if university-based to a local area network (modem or cable type). The slower of the two modems is the usual speed of connection. LONG: ISP connects to larger network service providers (NSP), the backbone of the Net using digital lines (T1). The data finds its way between computers through devices called ROUTERS. Information send through the Net is broken in small packets and routers function is to get those packets where they belong (IP address). It doesn't matter the path is takes to reach his destiny (> 10,000 packets/second). Real-time multimedia can suffer from router lassitude. The information in the Net originates within SERVERS - computers dedicated to serving data. PROTOCOLS are established to set how two computers communicate with each other. The TCP/IP protocol is the language of all Net computers. Protocols are embodied in BROWSER software (i.e. Netscape or MS Explorer) that help us navigate through the Net and are established by the Internet Engineering Task Force.
 

References
1- Wiggins R: How the Internet Works. Internet World October 1996 pag 54-60


Pancreatic Trauma

The pancreas fixed retroperitoneal location juxtaposed to the bony spine makes it vulnerable to minimal trauma. A direct blow (bike handlebar, fist or hockey stick) from blunt abdominal trauma can lead to contusion, laceration or transection of the organ. Clinically the child will develop abdominal pain, vomiting, and signs of peritoneal irritation. Pancreatic injury is most commonly recognized in trauma patients that are immediately explored due to hemodynamic instability, increase blood transfusion requirement, or hemorraghic shock associated to non-pancreatic injury. Other times there is isolated pancreatic injury identified by a rising amylase/lipase or after imaging studies. With the recent tendency toward conservative management of solid organ injury more reliance is placed on diagnostic clinical indicators (serum enzymes, ultrasound, CT-Scan findings, and ERCP) that help distinguish between minor and major injuries. Primary difficulty with conservative strategy is diagnosing major pancreatic injury. Untreated major pancreatic ductal injury leads to prolonged morbidity (longer stay from pancreatitis, pseudocyst formation, peripancreatic abscess formation, and post-traumatic ductal stricture). Minor injuries (contusion) have flat enzyme elevation with time and are managed conservatively with gut rest, NG decompression and TPN. Major injuries (gland transection, ductal laceration, and injuries resulting in pseudocyst formation) can be shown in CT-Scan, US, or ERCP; have consistently rising enzyme elevation and will need early surgical resection (distal pancreatectomy with splenic preservation), debridement a/o drainage.
 

References
1- Gorenstein A, Wesson DE, Schiller M: Pancreatic Trauma, In Schiller's Pediatric Surgery of the Liver, Pancreas and Spleen. WB Saunders Co. 1991 pag235-246
2- Synn AY, Mulvihill SJ, Fonkalsrud EW: Surgical Management of Pancreatitis in Childhood. J Pediatr Surg 22(7); 628-632, 1987
3- Gorenstein A, O'Halpin D, Wesson DE, et al: Blunt Injury to the Pancreas in Children: Selective Management Based on Ultrasound. J Pediatr Surg 22(12): 1110-1116, 1987
4- Bass Juan, Di Lorenzo M, Desjardins JG, et al: Blunt Pancreatic Injuries in Children: The Role of Percutaneous External Drainage in the Treatment of Pancreatic Psedocysts. J Pediatr Surg 23(8): 721-724, 1988
5- Vane DW, Grosfeld JL, West KW, et al: Pancreatic Disorders in Infancy and Childhood: Experince with 92 Cases. J Pediatr Surg 24(8): 771-776, 1989
6- Haller JA, Papa P, Drugas G, et al: Nonoperative Management of Solid Organ Injuries in Children Is it safe? Ann Surg 219(6): 625-631, 1994
7- Smith SD, Nakayama DK, Gantt N, et al: Pancreatic Injuries in Childhood Due to Blunt Trauma. J Pediatr Surg 23(7): 610-614, 1988
8- Rescorla FJ, Plumley DA, Sherman S, et al: The Efficacy of Early ERCP in Pediatric pancreatic Trauma. J Pediatr Surg 30(2): 336-340, 1995


Fistula-in-ano

Fistula-in-ano (FIA) practically occurs in male children during their first year of life. The pediatric incidence is low as judge by the scant literature. Most FIA has a single external orifice to either side of the anal canal, are superficial (low), communicates directly passing through the lowest fibers if the internal sphincter to open at the level of the anal valves. They are believed to be developmental anomaly caused by abnormal crypts of Morgagni that trap bacteria and initiates a cryptitis turning into either a perineal abscess or FIA. The perianal abscess is believed to be the parent if the FIA. 75% of males with FIA elicit history of perineal abscess. The presence of columnar, transitional and stratified squamous epithelium lining the tract is evidence of a congenital origin of FIA (entrapped migratory cells from the urogenital sinus development). FIA is managed with excision and laying opened the tract. Recurrences are common. Up to 15% of Crohn's children present with FIA.
 

References
1- Piazza DJ, Radhakrishnan J: Perianal abscess and fistula-in-ano in children. Dis Colon Rectum 33(12):1014-6, 1990
2- Levien DH, Surrell J, Mazier WP: Surgical treatment of anorectal fistula in patients with Crohn's disease. Surg Gynecol Obstet 169(2):133-6, 1989
3- Pople IK, Ralphs DN:  An aetiology for fistula in ano.Br J Surg 75(9):904-5, 1988
4- Fitzgerald RJ, Harding B, Ryan W: Fistula-in-ano in Childhood: A Congenital Etiology. J Ped Surg 20(1): 80-81, 1985
5- Shafer AD, McGlone TP, Flanagan RA: Abnormal Crypts of Morgagni: The Cause of Perineal Abcecess and Fistula-in-ano. J Ped Surg 22(3): 203-204, 1987
6- Brem H, Guttman FM, Laberge JM, et al: Congenital Anal Fistula with Normal Anus. J Ped Surg 24(2): 183-185, 1989
7- Palder SB, Shandling B, Bilik R, et al: Perianal Complications of Pediatric Crohn's Disease. J Ped Surg 26(5): 513-515, 1991
8- Al-Salem AH, Laing W, Talwalker V: Fistukla-in-ano in Infancy and Childhood. J Ped Surg 29(3): 436-438, 1994



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