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.

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.

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.

VOL 07 NO 02 AUGUST 1996

Meconium Ileus-like Syndrome

Meckel's Diverticulum

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.

VOL 07 NO 03 SEPTEMBER 1996

MMIHS

Intestinal Atresias

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

VOL 07 NO 04 OCTOBER 1996

Epignathus

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.

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.

VOL 07 NO 05 NOVEMBER 1996

Rhabdomyosarcoma

Hepatic Cysts

Henoch-Schönlein Purpura

VOL 07 NO 06 DECEMBER 1996

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.
 

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.
 

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.