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A newborn presenting with an isolated ectopic colonic tissue arising from the vulva without any associated anomaly is reported. The clinicopathologic features of this rare entity are discussed with special emphasis on embryologic basis.

A 13-year-old girl presenting with severe anemia was diagnosed to have a large gastric tumor protruding toward the antrum with two central ulcerations. Partial gastrectomy including antrectomy and gastroduodenostomy were performed. Histologic and immunohistochemical studies revealed one of the most uncommon gastric tumors in children; a gastrointestinal stromal tumor. Close follow-up of the patient with endoscopy, abdominal ultrasonography and/or computed tomography in three to six month intervals revealed no recurrences or metastasis of the tumor following its complete excision.

Gastroesophageal reflux associated with anatomic and functional abnormalities of the esophagus may be encountered following the repair of a congenital diaphragmatic hernia (CDH). We report herein the case of a newborn male infant with CDH found to have an air-filled paravertebral structure. Upper gastrointestinal series confirmed the presence of an ectatic esophagus with poor peristalsis and severe gastroesophageal reflux. The patient required jejunostomy after a safe period of total parenteral nutrition. He tolerated oral feeding following medical treatment without the need for antireflux surgery. The association of dysphagia, esophageal ectasia, and gastroesophageal reflux is rarely seen with CDH, and feeding intolerance is a self-limited disorder that does not usually necessitate antireflux surgery.

An unusual case of benign intrascrotal lipoblastoma simulating testicular torsion is presented, and the literature in English on the subject is reviewed.

Purpose: The aim of this study was to present an updated picture of surgical management of pediatric testicular tumors based on our 30 years' experience, which consisted of one of the largest noncollected series treated in a single medical center. Methods: Records of children who were treated for testicular tumor in our unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical history, clinical characteristics, diagnostic procedures, treatment methods, histopathologic findings, and outcome. Results: Fifty-one patients with a mean age of 3.8 ± 0.5 years were treated for testicular tumors. Of these, 35 (69%) had germ cell testis tumor (GCT) and 16 (31%) had non–germ cell testis tumor (NGCT). Endodermal sinus tumor and paratesticular rhabdomyosarcoma were the dominant histologic subtypes in each group, respectively. The most common mode of presentation was painless scrotal mass. At initial presentation, retroperitoneal (n = 5), both retroperitoneal and lung (n = 2), and retroperitoneal and liver (n = 3) metastases were recorded in 10 (19%) patients. Initial operative procedures were radical inguinal orchiectomy (RIO) (n = 29), scrotal orchiectomy (SO; n=9), bilateral RIO (n = 2), both RIO and unilateral retroperitoneal lymph node (RPLN) excision (n = 6), testis-sparing enucleation of the tumor (n = 5). SOs were performed elsewhere, and these patients underwent high ligation (n = 4) and both high ligation plus RPLN excision (n = 5) in our unit. Histopathologically, spermatic cord invasion and RPLN involvement were present in 10 patients. Scrotal recurrences were encountered in 2 patients who had scrotal orchiectomy initially. Retroperitoneal recurrences were noted in a patient presenting with stage I embryonal carcinoma and in 2 patients presenting with group IV paratesticular rhabdomyosarcoma. The mean follow-up period was 89 ± 10 months. Four patients with stage IV embryonal carcinoma (n = 2) and group IV paratesticular rhabdomyosarcoma (n = 2) died of progression of the disease. All remaining patients were alive and disease free at their last outpatient appointment. No significant difference was noted with regard to 5-year survival rates between (1) malignant GCT and paratesticular rhabdomyosarcoma patients (91% v 80%) and (2) patients treated by RIO (88%), SO plus high ligation (87%), and RIO plus RPLN excision (80%). Five-year survival rates were 100% for stage I, II, III patients and 33.3% for stage IV and group IV patients presenting with malignant testicular tumors ( P Conclusions: Childhood testicular tumors deserve special attention from the therapeutic point of the view. A solid scrotal mass should be considered malignant until proved otherwise. Any suspicion of the testicular tumor warrants an inguinal approach to prevent scrotal violation by the tumor. Current trends emphasize that testis-sparing surgery should be performed for benign lesions such as teratoma, leydig cell tumor, and epidermoid cyst based on frozen biopsy findings. Literature findings and our experience suggest that RIO is the accurate treatment for stage I malignant GCT and group I and IIa paratesticular rhabdomyosarcoma. RPLN excision is not of benefit either as a staging or therapeutic procedure in stage I and group I and IIa diseases of these tumors. RPLN excision should be reserved for (1) malignant GCT patients who have persistent elevation of alpha-fetoprotein after orchiectomy in the presence of normal total body CT scan, and for patients presenting with stage II and III disease with definitive abnormality on CT scans, and (2) group IIb, IIc, and III paratesticular rhabdomyosarcoma patients with radiologic evidence of retroperitoneal involvement on CT scans. High ligation should be done as a complementary procedure after SO to increase the survival rates. J Pediatr Surg 36:1796-1801. Copyright © 2001 by W.B. Saunders Company.

Evidence suggests differences in contractility in cremaster muscles (CM) associated with undescended testis caused by alterations of autonomic innervation. Contractile responses of CM to various pharmacologic agents were evaluated and compared according to the localization of testis.Samples of CM from boys with undescended testis or inguinal hernia were obtained. Twitch and tetanic contractions were recorded isometrically at 37 degrees C. Effects of verapamil, isoprenaline, calcitonin gene-related peptide (CGRP), substance P (SP) and N(omega)-nitro-L-arginine (L-NNA) were investigated. Results were compared through 2-way analysis of variance, and P values less than.05 were considered to be different.Verapamil alone significantly (P.05) decreased contraction amplitudes in CM from both sources; the decrease was more pronounced in CM from boys with inguinal hernia (P.05). Although isoprenaline increased contraction amplitudes in CM associated with undescended testis (P.05), CGRP and SP increased contraction amplitudes in CM associated with descended testis (P.05). L-NNA increased contraction amplitudes in both groups (P.05). The decrease of contraction amplitudes after verapamil displayed a similar pattern after isoprenaline, SP, and L-NNA. Verapamil-induced contractility decrease was more pronounced after CGRP in both groups (P.05).Sensitivity of CM to verapamil differs according to localization of testis. Isoprenaline enhances contractility by stimulating Na(+)-K(+)ATPase in undescended testis without altering voltage-sensitive channel sensitivity to verapamil. CGRP and SP increase contractility in inguinal hernia, and CGRP increases the sensitivity of voltage-sensitive Ca(2+) channels to verapamil in CM from both groups. Nitric Oxide (NO) exerts inhibitory action on CM contractility, and it is less pronounced in undescended testis. These differences may contribute to pathophysiology of undescended testis.

Introduction: Inguinal hernia and hydrocele are suggested to result from the persistence of smooth muscle (SM) which should undergo programmed cell death (PCD) after presenting transiently to propel the testis. Since Ca2+ is involved in PCD, the Ca2+ contents of the peritoneum and sacs associated with undescended testis, inguinal hernia and hydrocele were determined and compared. Materials and Methods: Sacs were obtained from boys with undescended testis (n = 11), inguinal hernia (n = 22) and hydrocele (n = 10), and girls with inguinal hernia (n = 7). The calcium content of the sacs and peritoneal samples (n = 6) was determined through atomic absorption spectrophotometry. Calcium contents were compared according to their sources using the Mann-Whitney U test and p values of Results: While revealing similar Ca2+ contents as the peritoneum, sacs associated with undescended testis and hydrocele contained more Ca2+ contents than the sacs of boys and girls with inguinal hernia (p < 0.05). Conclusions: Sacs associated with inguinal hernia, which are known to contain SM all around the mesothelial layer, contain the least Ca2+. Despite the decrease in SM, sacs associated with hydrocele contain more Ca2+. Since PCD is associated with Ca2+ overload and inhibition of Ca2+ load inhibits PCD, differences in Ca2+ content may reflect the inhibition of PCD at different stages and for different reasons in inguinal hernia or hydrocele of childhood.

Skeletal metastases is relatively rare in hepatocellular carcinoma and accounts for 4-16% of extrahepatic metastases. The authors report a 13-year-old girl with fibrolamellar hepatocellular carcinoma, who rejected further systemic chemotherapy following hepatic lobectomy and experienced sternal and vertebral painful metastases nearly 5 years after the operation. The sternal metastatic lesion was removed surgically, whereas external irradiation was delivered to the lumbar vertebral lesion. The patient received no systemic treatment following metastases and died with widespread disease. Despite metastatic disease, the patient survived 6.5 years following the initial diagnosis.

Background/Purpose: The cremaster muscles (CM) associated with undescended testis reveal neurogenic alterations that mainly affect type 2 fibers. The ultrastructure of CM has been evaluated to define if further evidence to explain the alterations could be identified. Methods: CM of 8 boys with inguinal hernia and 8 boys with undescended testis at similar ages were biopsied. Samples were processed for electron microscopic evaluations. Semithin and thin sections were examined under an electron microscope. Results: The CM associated with inguinal hernia showed normal ultrastructure. However, some alterations were encountered in CM associated with undescended testis. Unmyelinated fibers were diminished in number, and myelinated fibers were outnumbering the unmyelinated fibers. Marked disorientation of myofibers, redundant sarcolemma, empty sleeves of basal lamina, disarray of myofibrils, densely packed myofilaments, Z disk streaming, dilated sarcoplasmic reticulum, and dense-irregularly shaped mitochondria were repeatedly encountered. Satellite cells appeared inactive. Most of the fibers were contracted. Conclusions: The decrease in number of unmyelinated fibers appears to represent a decrease in autonomic nerve fibers. The alterations within muscle fibers may reflect a deficiency in autonomic innervation. Autonomic nervous system is highly responsive to circulating androgens. Factors decreasing the vulnerability of autonomic nervous system against androgenic effects may result in a CM with neurogenic alterations, thus inhibiting testicular descent. J Pediatr Surg 36:573-578. Copyright © 2001 by W.B. Saunders Company.

Background/Purpose: Etiopathogenesis and management of pediatric adrenocortical tumors (ACTs) is still obscure because of the limited number of cases. The aim of this study is to present a clear picture of the entire spectrum of pediatric ACTs by reviewing one of the largest noncollected pediatric series treated in a single medical center. Methods: Records of children treated for ACTs in our unit between 1970 and 1999, inclusive, were reviewed. Information recorded for each patient included age, sex, clinical characteristics, diagnostic methods, stage of disease, treatment, pathologic findings, and outcome. The patients were subdivided into 2 groups: group I, patients with adrenocortical carcinoma (ACC) and group II, patients with adrenocortical adenoma (ACA). These groups were analyzed with regard to parameters mentioned above. Results: There were 30 children treated for ACTs in the study period with a mean age of 6.7 ± 4.2 years (range, 2.5 to 13 years). Of these, 20 had ACC, and 10 had ACA. The tumors were right sided in 22 patients, left sided in 6 and bilateral in 2. Analysis of each group with regard to age and site of tumor showed no significant difference. Endocrine dysfunction was noted in 83% of the patients and virilization was the most common presentation followed by Cushing's syndrome. The most striking difference between 2 groups was the prepondarance of virilization in group II and Cushing's syndrome in group I. In the latter, 14 patients presented with palpable abdominal mass and 3 patients with distant metastases. The mean time from initial symptoms to diagnosis was 8.1 ± 0.2 months, and this interval was similar in 2 groups, in functional and nonfunctional tumors, and in both sexes. Ultrasound scan, computerized tomography, magnetic resonance imaging, intravenous pyelography, and angiography were used for the diagnosis. All patients with ACA had localized disease, whereas 80% of the patients with ACC had regional or metastatic disease. Total excision was done in all patients with ACA, but only in 13 patients with ACCs. Of the latter, 2 patients underwent ipsilateral nephrectomy, and 1 patient had right hepatic lobectomy plus nephrectomy. Adjuvant chemotherapy consisting of mitotane (n = 12), mitotane plus cisplatin and etoposide (n = 2) was commenced. Seven patients with ACC had distant metastases postoperatively. The presence of regional disease at presentation was associated with a significantly shorter disease-free interval. All patients presenting with nonfunctional ACC (n = 4), functional ACC that have been totally resected (n = 4), and partially resected (n = 3) died of disease within the first 2.5 years after diagnosis. There was no significant difference between the functional and nonfunctional ACCs with regard to survival rate. All patients who had distant metastases postoperatively and who had partial excision died. Of the surviving 9 patients with ACC, there are 6 known long-term survivors who are still alive. Conclusions: ACAs are treated by total excision satisfactorily without any complication. For the time being, the most important aspect of therapy for ACCs is early diagnosis and total excision. Partial excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy in ACTs. Because of the steadily increasing incidence of precancerous genetic syndromes of adrenal glands and poor prognosis of ACCs, childhood patients of endocrine disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults. Patients with ACTs should be entered into multi-institutional trials to adequately assess effective chemotherapy and radiotherapy protocols and molecular mechanisms of oncogenesis. J Pediatr Surg 36:549-554. Copyright © 2001 by W.B. Saunders Company.

Background/Purpose: Etiopathogenesis, management, and outcome of pediatric pheochromocytoma (PHEO) still is obscure because of limited number of cases. Therefore, a retrospective clinical study was performed to present an updated picture of the entire spectrum of pediatric PHEO based on the authors' 30 years' experience consisting of one of the largest noncollected series treated in a single medical center. Methods: Records of patients treated for PHEO in the authors' unit from 1970 to 1999, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, past medical and family history, clinical characteristics, diagnostic methods, treatment, pathologic findings, and outcome. Results: Sixteen children with a mean age of 10.7 ± 2.9 years consisting of 12 boys and 4 girls were treated for PHEO. Most of the tumors were right sided (n = 6) and bilateral (n = 6). Sporadic cases of PHEO accounted for 14 patients (88%), whereas 2 children had von Hippel-Lindau (VHL) disease and multiple endocrine neoplasia type 2b (MEN2b). Hypertension was the most common symptom followed by headache and sweating. The diagnosis of PHEO was made by laboratory and radiologic studies. Preoperative medical therapy was done in all patients. Laparotomy confirmed that 11 patients had localized, 4 patients had regional, and 1 patient had metastatic disease. The localized tumors were excised totally by bilateral (n = 4) and unilateral (n = 6) adrenalectomy. Surgical procedures performed for regional disease were total excision (n = 2), incisional biopsy (n = 1) and partial excision (n = 1). Incisional biopsy could be taken only from a patient with metastatic disease at presentation. Two patients with localized disease and 2 patients with regional disease had benign recurrences in right (n = 2) and left (n = 2) adrenal glands within 3 to 7 years after operation. Total excision of the recurrent tumors was done in all patients. Pathologic examination found apparently malignant features in 3 patients who presented with regional (n = 2) or metastatic (n = 1) disease and underwent incisional biopsy (n = 2) or partial excision (n = 1). Pathologic features suggestive of malignancy were noted in 4 patients presenting with regional (n = 2) and localized disease (n = 2). Apparently benign pathologic features were noted in the remaining 9 patients. There was not any operative mortality in our series. Adjuvant chemotherapy was commenced postoperatively in all patients with malignant and suggestive of malignant pathologic features. During the long-term follow-up for 16 years, 3 patients died (19%). One patient with VHL disease died of astrositoma 5 years after her recurrent PHEO was excised. Of the 3 patients with malignant disease, 2 patients in whom only incisional biopsies were done had distant metastases and died of disease within 2 years. Another patient with malignancy who had MEN2b was lost to follow-up. Conclusions: Early diagnosis and total excision are the most important aspects of accurate treatment for childhood PHEO. Pre- intra- and postoperative medical management is as important as the surgical procedure. Our surgical treatment policy is mainly minimizing the risk of recurrence while preserving adequately functioning adrenal medullar tissue. Incomplete excision and advanced-stage disease are the major determinants of poor outcome. None of the clinical, laboratory, or pathologic features are reliable predictors for recurrence and discrimination of malignancy. Because of the steadily increasing incidence of precancerous genetic syndromes related to adrenal glands and poor prognosis of advanced-stage PHEO, childhood cases of hypertensive disorders should receive a detailed and vigorous diagnostic evaluation and appropriate treatment as given to adults. J Pediatr Surg 36:447-452. Copyright © 2001 by W.B. Saunders Company.

Introduction A retrospective clinical study was carried out to define the appropriate definitive operation for treating differentiated thyroid carcinoma (DTC) in children. Methods During a 24-year period between 1974 and 1997, 18 children younger than 16 years at presentation were histopathologically proven to have DTC in Hacettepe University Children's Hospital, Department of Pediatric Surgery. The clinical characteristics on admission, operative findings, procedures, operative complications, and follow-up results according to definitive operations were compared to find out the appropriate procedure. Results There were 15 female (83.3%) and 3 male (16.7%) patients yielding a female/male ratio of 5 and the average age being 11.6+/-2.1 years. The definitive operations were near total thyroidectomy, total thyroidectomy, near total thyroidectomy plus excision of the only palpable lymph nodes (EPLN), total thyroidectomy plus EPLN and total thyroidectomy plus modified radical neck dissection (MRND) in 3 (16.7%), 3 (16.7%), 1 (5.5%), 9 (50%), and 2 (11.1 %) patients respectively. Among the patients having undergone near total thyroidectomy, one had recurrences both in the thyroid bed and the cervical lymph nodes. In a patient undergoing almost total thyroidectomy and EPLN, recurrences involving thyroid bed, cervical lymph nodes and lungs were encountered. In 9 patients undergoing total thyroidectomy and EPLN 4 had cervical lymph node recurrences. Complications have included permanent hypoparathyroidism following total thyroidectomy and MRND. Additionally, operations for recurrences resulted in unilateral permanent recurrent laryngeal nerve palsies in two patients. Conclusions Total thyroidectomy with evaluation of central compartment, entire jugular chain and region of lower spinal accessory nerve and removal of all identifiable lymph nodes seems to be the most appropriate surgical treatment for DTC encountered in children.

Objective: To evaluate the effect of capsaicin, a powerful neurotoxin selective to afferent nerves, on contralateral testicular damage in ipsilateral testicular torsion. Methods: Forty male albino rats were randomly allocated into five groups. No operation was performed in group one. After intraperitoneal administration of 0.9% NaCl, rats underwent a sham operation in group 2 and testicular torsion in group 3. In groups 4 and 5 rats underwent sham operation and testicular torsion, respectively after intraoperitoneal capsaicin injection. Contralateral testes were harvested on the fifteenth day of the experiment and mean seminiferous tubular diameters and mean testicular biopsy scores were recorded for each testis. The values were compared through analysis of variance (ANOVA) with Turkey-Kramer multiple comparisons test and p values less than 0.05 were considered to be significant. Results: Mean testicular biopsy scores and mean seminiferous tubular diameters of group 5 was significantly higher than the group 3. There was no difference between the groups 1, 2, 4, and 5 when these two parameters are concerned. Conclusion: Capsaicin effectively prevents contralateral testicular damage encountered following ipsilateral testicular torsion. The inhibition of afferent impulses from the ipsilateral testis under distress prevents contralateral testicular injury, and provides additional data to support the role of an autonomic reflex arc in contralateral testicular injury. Copyright © 2001 S. Karger AG, Basel.

The records of eight patients treated for intestinal obstruction resulting from bands that have no identifiable embryologic or acquired basis were reviewed retrospectively. All patients presented with symptoms and signs indicative of intestinal obstruction. Patients older than 2 years of age additionally had a history of chronic abdominal pain. One thick anomalous congenital band with blood vessels in it was found to be the cause of obstruction in each patient. Bands were located between ascending colon and terminal ileum in four patients (50%), ligament of Treitz and terminal ileum in two patients (25%), right lobe of liver and terminal ileum in one patient (12.5%), and right lobe of liver and ascending colon in one patient (12.5%). The obstructive mechanisms were compression of bowel by band in five patients (62.5%) and entrappment of an intestinal loop between the band and mesenterium in three patients (37.5%). These bands are suggested to be the anomalies of mesenterium that may cause intestinal obstruction and chronic abdominal pain in children.

The records of 228 episodes of adhesive small-bowel obstruction (ASBO) in 179 children were reviewed retrospectively to evaluate criteria to predict the presence of vascular compromise of the intestine and to establish safety limits for conservative treatment. Among 228 episodes of ASBO, 16 (7%) were associated with vascular compromise while 212 (93%) were without. The predictability of the proposed criteria, which included localized abdominal tenderness, tachycardia, fever, and leukocytosis was studied individually and in combination. Of the individual criteria, the presence of tachycaria and localized abdominal tenderness correlated significantly with the presence of vascular compromise (P

Colonic atresia (CA) is a very rare cause of intestinal obstruction, and little information has been available about the management and predictors of outcome. A retrospective clinical trial was performed to delineate the clinical characteristics of CA with special emphasis on surgical treatment and factors affecting outcome. Children with CA who were treated in our department between 1977 and 1998 were reviewed: 14 boys and 4 girls aged 1 day to 5 months. All but 2 referred patients and 1 with prenatal diagnosis presented with intestinal obstruction. Plain abdominal X-ray films showed findings of intestinal obstruction in 14 cases; a barium enema demonstrated a distal atretic segment and microcolon in 4. The types of atresia were IIIa (n=9), I (n=6), and II (n=3). Type IIIa atresias were located proximal to the splenic flexure (n=8) and in the sigmoid colon (n=1), type I atresias were encountered throughout the colon; and all type II atresias were proximal to the hepatic flexure. Associated anomalies were multiple small-intestinal atresias (MSIA) (n=4), gastroschisis (GS) (n=2), pyloric atresia (n=1), Hirschsprung's disease (n=1), and complex urologic abnormalities (n=1). The initial management was an enterostomy in 15 patients (83%), including 2 referred and 2 with GS, and primary anastomosis in the remaining 3 (17%). Secondary procedures were the Santulli operation (n=2), colostomy closure and recolostomy followed by a Swenson operation (n=1), sacroabdominoperineal pull-through (n=1), and colostomy closure (n=1). Leakage was encountered in all primarily anastomosed patients. The overall mortality was 61%. Deaths occurred in patients with associated major anomalies (GS 2, MSIA 3, pyloric atresia 1) (55%) and in 3 patients who were initially managed by primary anastomosis (27%). Two additional patients died of sudden infant death syndrome (18%). Type I atresia was more common than in previously reported series and was associated with proximal multiple atresias. The initial management of CA should be prompt decompression of the intestine by an ostomy procedure, preferably end- or double-barrel. The type of surgery (primary anastomosis without prior colostomy) and associated abnormalities are the major determinants of poor outcome.

Summary In the present study, we determined and compared the distribution and mean diameters of fiber in the cremaster muscles (CM) of boys with either inguinal hernia (IH) or undescended testis (UT). Samples of CM were obtained from 20 patients (10 boys with IH, and 10 boys with UT) of similar age. The CM muscles of two boys each, without inguinal pathology, were sampled during autopsy. Sections were stained for oxidative and glycolytic enzymes, as well as for ATP-ase reactions after acid (pH: 4.6) and alkaline (pH: 10.6) preincubations. Specimens were also analyzed morphometrically using a KONTRON 400 computerized image analysis system. The Mann- Whitney U test was applied to compare the percentages of fiber types and mean diameters of fibers according to the types of the CM of boys with IH or UT. In boys, the CM is mainly composed of type 1 fibers. The CMs of patients with UT reveal alterations of neurogenic origin. Although both type 1 and type 2 fibers reveal alterations, type 2 fibers appear to be affected more profoundly and characterized by significantly decreased mean diameters. Significantly decreased mean diameters of type 2 fibers in CM may support disuse, lack of sensitivity to the hormonal influences, or an alteration in the corticospinal tracts of boys with UT.

Purpose: The records of 4 patients who had necrotic bowel secondary to acute mesenteric vascular occlusion affecting various levels of mesenteric vasculature were reviewed to determine the clinical manifestations, diagnostic investigations, predisposing factors, complications, and outcome of mesenteric vascular thrombosis in children. Methods: The medical records of the patients (3 boys, 1 girl) treated between 1981 and 1996, inclusive, for bowel infarction secondary to mesenteric vascular thrombosis, were reviewed with regard to signs and symptoms, laboratory tests, radiological investigations, surgical findings, histopathologic examinations, and outcome. Results: The ages of the patients ranged between 1 and 14 years with a mean age of 8.2 years. Initial symptoms, present in all patients, were abdominal pain, abdominal distension, and tenderness. Laboratory and radiological findings including abdominal radiographs and abdominal ultrasonography were nondiagnostic. Selective superior mesenteric angiography showed complete obliteration of the superior mesenteric artery with absence of venous return in 1 case. Three patients with massive intestinal necrosis died of multiorgan failure or the complications of short bowel syndrome. Histological examination of the resected intestinal segments showed the typical findings of polyarteritis nodosa in 2 patients. One patient had a previous history of right femoral vein thrombosis, whereas 1 patient had no known underlying disorders predisposing vascular thrombosis. Conclusions: Mesenteric vascular occlusion is a rare but serious disease leading to death in children. The patients present with similar clinical signs, most frequent and important are acute abdominal pain, vomiting, and distension. Mesenteric vascular occlusion is a rare cause of acute abdomen in childhood, which requires urgent diagnosis and intervention. In suspected mesenteric vascular insufficiency, angiography should be performed followed by intraarterial thrombolytic infusion therapy in selected cases. When intestinal infarction is suspected, immediate surgical resection of compromised bowel is necessary with appropriate postoperative anticoagulation or treatment of any underlying disease. J Pediatr Surg 35:1161-1164. Copyright © 2000 by W.B. Saunders Company.

A 10-year-old girl has experienced 3 recurrences of hepatic artery thrombosis (HAT) after a liver transplantation. She responded to intraarterial administrations of urokinase after the first 2 attacks. However, the restoration of the arterial flow was not possible after the third attack. The child and her father were both heterozygous for factor V Leiden mutation. In addition to the technical factors, the factor V Leiden mutation should be considered as a factor that plays a role in HAT.

Objective To evaluate the effects of increased intra-abdominal pressure (IAP) on the contractility of the rabbit bladder, as the dynamics of the bladder may be impaired in conditions associated with a high IAP, e.g. constipation and pregnancy. Material and methods The study comprised 22 adult male New Zealand rabbits; six served as the control group, eight had an IAP of 7 cmH2O imposed for 10 days by instilling air into the abdominal cavity and this IAP was maintained for 60 days in a further eight rabbits. After treatment, the rabbits were killed, and the bladders removed and cut into 3 × 12 mm strips. The contractile activity of the muscle strips was then recorded isometrically. Electrical field stimulation (EFS) was applied using a pair of platinum ring electrodes in trains of 3 s duration every 100 s (1 ms, 100 V, 2–100 Hz). Contractile responses to carbachol and isotonic KCl were also evaluated. Results EFS induced a frequency-dependent increase in contractile activity in all bladder strips. Ten days of high IAP resulted in an increased responsiveness to EFS, but high IAP for 60 days reduced the EFS-induced responses to the control levels. Carbachol (10−9–10−3 mol/L) elicited concentration-dependent contractions in all groups. From the concentration-response curves of carbachol, the log EC50 values (the concentration producing half the maximum effect) of the control and 60-day treated animals were comparable, at −6.24 (0.05) and −6.25 (0.04), respectively. However, the log EC50 of the 10 day-treated group was −4.97 (0.08) and significantly (P

L'ectopie de muqueuse gastrique dans la partie superieure de l'oesophage a deja ete rapportee dans des autopsies neonatales et rencontrees au cours des œsophagoscopies chez l'adulte. Habituellement, cette anomalie est asymptomatique mais des cas cliniques symptomatiques ont ete rapportes chez l'adulte. Cependant, il n'y a pas d'observation d'enfant porteur d'ectopie de muqueuse gastrique symptomatique dans la litterature. Les auteurs rapportent l'observation d'un garcon de 12 ans qui presentait une dysphagie severe due a un retrecissement consecutif a des inclusions circulaires de muqueuse gastrique situees au niveau de l'oesophage moyen.

An experimental study was performed to investigate the gastrointestinal motility and in-utero defecation by radionuclide techniques. Forty-eight New-Zealand white rabbit fetuses at 25 days' gestation (fullterm, 31 to 32 days) were divided into two groups as A (n: 24) and B (n: 24). 0.1 ml of Technetium-99m (99mTC)-HIDA (a derivative of iminodiacetic acid) containing 1 mCi of radioactivity was injected into the gluteus muscle of each fetus, which had been exposed through the uterus. This procedure was used for all fetuses and additionally surgical closure of anus by a purse string suture was performed in Group B fetuses. After replacing the fetus and uterus into the abdomen, and beginning 1 hour after injection, a live fetus was killed each hour for 24 hours. Tissue samples from the reference organs (lung, heart, stomach, kidney, bladder), liver, meconium in the proximal, mid and distal bowel, and amniotic fluid were taken. The radioactivity of each sample was determined by a gamma counter and the percentage injected dose (uptake) per gram of tissue is calculated. 99mTc-HIDA was predominantly trapped by the liver via systemic circulation and excreted into the gastrointestinal tract in both groups through which it passed into the amniotic fluid only in Group A. The very low radioactivity levels detected in the amniotic fluid of Group B originated from urinary tract and indicated the in-vivo stability of 99mTc-HIDA, thus reliability of the experimental model. Intestinal transport of a radiopharmaceutical agent in both groups and demonstrated passage into the amniotic fluid only in Group A strongly suggest that fetal defecation is a physiologic event.

The first childhood case of splenic hemangiopericytoma in a 10-year-old boy is presented. The clinicopathologic features of this unique entity are discussed with special emphasis on differential diagnosis and treatment by comparing with the previously reported adult cases. There are no specific presumptive clinical and laboratory findings, including tumor markers and imaging techniques that distinguish hemangiopericytoma from other splenic masses. The most important diagnostic aid is to bear this entity in mind when a child presents with an unexplained splenic mass. Splenectomy associated with chemotherapy or radiotherapy in the presence of systemic or local recurrences is mandatory for the appropriate treatment.

Objective To compare cremaster muscles (CMs) obtained from boys with inguinal hernia, hydrocele or an undescended testis and those obtained from girls with inguinal hernia, thus defining the changes associated with each clinical condition. Materials and methods CM samples were obtained from 26 boys and three girls with inguinal hernia, and 18 boys who had undergone surgery for an undescended testis (12) or hydrocele (six). The samples were frozen in isopentane cooled in liquid nitrogen and were processed for sectioning by cryostat. Sections (12 µm) were stained with a several histochemical stains. The presence of central nuclei, fibre splitting, basophilic fibres, fibre necrosis, inflammatory changes, small angular fibres, fibre hypertrophy, grouped atrophy, and endo- and perimysial fibrosis were evaluated. From each specimen, 200 fibres were also analysed morphometrically using a computerized image analysis system. Results Neurogenic changes were apparent in all the CMs from patients with an undescended testis but none of the samples obtained from girls showed any changes. While only two specimens of 26 from boys with inguinal hernia (8%) had evidence of neurological alterations, eight CM (31%) had general changes. The mean (SD) fibre diameters did not differ significantly among the groups with inguinal hernia, hydrocele and undescended testis, at 23.0 (8.6), 24.4 (4.5) and 23.0 (10.5) µm, respectively. Conclusion Cremasteric muscles associated with an inguinal hernia or an undescended testis differ; neurogenic changes were detected within all the CM of boys with an undescended testis. These changes in the CM may have influenced the location of the testis.

Intussusception is one of the most common causes of bowel obstruction in infancy which may require surgical treatment. We have recently recognized a pathologically weakened linear area located on the wall of the reduced colon at operation. Thus, a retrospective study was conducted to evaluate the operative and pathological findings of the resected bowel segments for the treatment of intussusception. A pathologically weakened longitudinal linear area was encountered in five patients. This line was strikingly antimesenteric and under the taenia libera. The bowel wall was very thin and effaced on palpation along this whitish line and showed mucosal necrosis, disruption of the muscularis mucosa, and loss of some of the muscular tissue on microscopic examination. The line may result from compression of the inner layers of the bowel wall between the intussusceptum and the noncompliant taenia. Location on the antimesenteric border and under the taenia libera can be explained by local vascular compromise due to the distribution of the terminal arteries of the colon. Thus, the antimesenteric border as well as the mesenteric side should be checked carefully for a longitudinal weakened pressure line. The recognition of such a potentially dangerous weak line on the bowel wall indicates resection.

Background/Purpose: Colorectal carcinoma is extremely rare in children and presents with a poor prognosis. Surgical management and long-term follow-up of this entity are still obscure because of lack of data. Therefore, a retrospective clinical trial was performed to evaluate the clinical characteristics of childhood colorectal carcinoma and to determine the predictors of poor outcome. Methods: Records of children who had colorectal carcinoma and were treated at our unit between 1972 and 1997, inclusive, were reviewed retrospectively. Information recorded for each patient included age, sex, clinical characteristics, diagnostic procedures, extent of disease, treatment methods, histological types, and outcome. A modified Dukes staging scheme was used. Results: There were 12 boys and 8 girls who were treated for colorectal carcinoma (range, 7 to 16 years). Predisposing conditions were encountered in 2 patients (10%), one with Bloom's syndrome and another with familial occurrence of colonic carcinomas. Predominant symptoms were abdominal pain, vomiting, and rectal bleeding. Barium enema, ultrasonography, computerized tomography, and endoscopic procedures were used for the diagnosis. Rectosigmoid region was the most common site for the primary tumor (65%). All patients presented with advanced stages of disease (stage C, 7; stage D, 13). Surgical procedures were incisional biopsy (n = 4), palliative permanent colostomy (n = 4), segmental resection (n = 5), complete resection (n = 6), and rectal biopsy (n = 1). Peritoneum was the most common site of extensive intraabdominal disease followed by omentum majus and liver. The lung also was involved in 2 patients. Predominant histological type was mucinous adenocarcinoma (80%). All patients but one received adjuvant chemotherapy, and 2 received palliative radiotherapy. Thirteen patients died of disease in a period ranging from 1 day to 1 year after initial surgery. The fate of 4 patients who were discharged in a very ill status was unknown. Three patients were alive for 2 years to 4 years postoperatively, and one of them presented with end stage disease. Conclusions: Delayed diagnosis, advanced stages of disease at presentation, and, most importantly, mucinous type of histology are the major determinants of poor outcome in childhood colorectal carcinoma. We emphasize that possibility of a malignant colorectal tumor should be considered for any childhood case with signs and symptoms of intestinal obstruction, intractable abdominal pain, alteration in bowel habits and gastrointestinal bleeding. Colorectal malignancy should not be excluded only on the basis of the patient's age. Because of the steadily increasing incidence of precancerous bowel diseases and poor prognosis of colorectal carcinoma, childhood cases of bowel disorders should receive the same detailed and vigorous diagnostic evaluation and appropriate treatment as given to adult cases. Contrast studies, ultrasonography, computed tomography, and endoscopy are essential procedures for both confirming the diagnosis and detecting the extent of the disease.

Background/Purpose: Congenital lobar emphysema (CLE) is characterized by overinflation of a pulmonary lobe and may present as a diagnostic and therapeutic dilemma. The authors have reviewed their experience to clarfy the controversies about the diagnosis and management of CLE in children. Methods: Children who had CLE and were treated at our department between 1991 and 1998, inclusive, were reviewed. Results: Fourteen children consisting of 8 boys and 6 girls (age range, 25 days to 2.5 years) had CLE. Eight children (57%) were symptomatic at birth. Major presenting symptoms were dyspnea (n = 8, 57%), cyanosis (n = 6, 43%) and recurrent respiratory tract infection (n = 4, 28.5%). Chest x-rays and computerized tomography scans showed hyperaeration of the affected lobes in 14 and 12 patients, respectively. Pulmonary perfusion scan was performed in 8 patients and showed loss of perfusion in the affected lobe in 7 cases. The affected sites were left upper lobe (n = 8, 57%), right middle lobe (n = 4, 28.5%) and right upper lobe (n = 2, 14.5%). Congenital cardiac anomalies were encountered in 2 patients (14.5%). Ten children underwent lobectomy, and postoperative course was uneventful. Nonoperative management was performed in 4 cases (28.5%) presenting at older age with milder symptoms. In the latter, although symptoms subsided, radiological abnormalities persisted during the follow-up period of 3 months to 4 years. No mortality was encountered in the series. Conclusions: The diagnosis of CLE is established by combined radiological and scintigraphic imaging methods. Surgical excision of the affected lobe is the appropriate treatment in all infants under 2 months of age and in infants older than 2 months presenting with severe respiratory symptoms. Infants older than 2 months presenting with mild to moderate respiratory symptoms associated with normal bronchoscopic findings can be treated conservatively. In conservative management, the children should be under close follow-up, and the family should be alarmed about the disease. Further studies are required to determine the long-term outcome of conservative management.

Pulmonary blastoma (PB) is an extremely uncommon intrathoracic neoplasm in children. It accounts for 0.5% of all primary malignant tumors of the lung, and 20%–25% of the cases present in childhood. A new case of PB in a 3-year-old girl is discussed with special emphasis on diagnosis and treatment. The authors point out that the possibility of a primary lung tumor should be considered for any childhood cases with intractable signs and symptoms of a space-occupying lesion. A primary lung malignancy should not be excluded only on the basis of the patient's age, and childhood lung disorders should receive the same detailed and vigorous diagnostic evaluation and appropriate treatment given to adults.

Background/Purpose: Histological structures of peritoneum, processus vaginalis, and sacs obtained from girls with inguinal hernia and boys with inguinal hernia, hydrocele, and undescended testis have been compared through immunohistochemical features to evaluate if any clue descriptive for the etiology of inguinal hernia exists. Methods: Parietal peritoneums (n = 6), processus vaginalises (n = 4), female hernia sacs (n = 5), male hernia sacs (n = 12), and sacs from hydrocele (n = 5) and undescended testis (n = 9) were stained with indirect immunoperoxidase method. Anti-CD9, CD26, CD29, CD31, CD36, CD44, CD49a, CD49b, CD49c, CD49d, CD49e, CD49f, CD54, CD55, CD56, CD62E & P, CD71, CD98, CD102, CD106, CD146, CD151 monoclonals and NFL-NPH, S-100 antiserums were used. The histological structures of each group of samples were identified and compared. Results: Smooth muscle layers have been encountered within the walls of hernia sacs of both boys and girls. Although the hydrocele sacs have shown smooth muscle bundles distributed as patchy areas, smooth muscle bundles have been observed infrequently among sacs from patients with undescended testis. Peritoneum and processus vaginalis samples have been free of smooth muscle. Conclusions: Inguinal hernia during childhood seems to be related to the presence of smooth muscle within the wall of the sac. The smooth muscle bundles may have played a role both in prevention of obliteration and clinical outcome. Because the sacs associated with undescended testis are without smooth muscles, and herniation is not a frequent association, they may not share the same etiologic basis with inguinal hernia.

A retrospective clinical study was performed to determine the incidence, management, and outcome of gastric outlet obstruction (GOO) caused by caustic ingestion in children. Of 220 patients who sustained caustic substance ingestion and were treated at our unit between 1976 and 1996, 168 ingested alkaline substances; of these, 9 children (5.3%) developed GOO in addition to esophageal strictures. The remaining 52 patients ingested acid agents, and 2 of them (3.8%) presented with GOO without esophageal strictures. The overall incidence of corrosive GOO was 5% (n = 11). The mean age of the patients with GOO was 5.7 +/- 2.8 years (range 2-14) with a female:male ratio of 6:5. Sodium hydroxide (n = 6), potassium hydroxide (n = 3), and hydrochloric acid (n = 2) were the ingested caustic agents. The patients were subdivided into two groups according to serial endoscopic and radiologic findings: group I: moderate (dense superficial and spotty ulcerations with intact mucosa) mucosal injury with partial pyloric obstruction; and group II: severe (deep ulcerations, extreme hemorrhagic erosions, eschar formation with white plaques) mucosal injury with complete pyloric obstruction. Group I consisted of 5 patients who ingested alkali agents while group II included 6 who presented with ingestion of alkaline (n = 4) and acid (n = 2) agents. Surgical treatment included Billroth I (n = 6) operations performed in group II and Finney (n = 3) and Heineke-Mikulicz (n = 2) pyloroplasty procedures done in group I. All patients are alive without any complaints. Fiberoptic endoscopy should be the preferred method of evaluating a patient with ingestion of a corrosive agent. It determines the presence of injury and assesses the extent of damage, establishing the diagnosis and allowing therapy to be instituted immediately. Our experience revealed that substantial damage has occurred early after ingestion, and early surgical intervention has decreased the morbidity and mortality. The extent of the mucosal injury and status of the pylorus and antrum determined the type of surgical treatment. A Billroth I procedure recommended for severely injured mucosa with complete pyloric obstruction, and pyloroplasty for moderate mucosal injury associated with partially obstructed but still viable pylorus. In contrast to the current belief, alkali ingestion also has a high risk of corrosive gastric injury causing GOO, which should be considered during assessment of the injury. We emphasize that a detailed evaluation of radiologic and especially endoscopic findings is very important for determining the timing, necessity, and type of appropriate surgical treatment.

A rare case of congenital urethral atresia with urethrorectal communication is presented. The embryological misdevelopment and one-stage correction of the anomaly are discussed.

Impairment of the vascular supply to a segment of bowel may be one of a number of causes of intestinal atresia. Intrauterine intussusception is an extremely rare cause of intestinal atresia. The factors responsible for this disorder are still obscure. According to our knowledge, no specific leading point has been reported as yet. In some cases a polypoid mass has been found within the post-atretic segment of bowel and has been considered a remnant of intussusceptum. We report a type IIIa ileal atresia due to intrauterine intussusception with a Meckel's diverticulum within the post-atretic intestinal segment; this is the first case reported in the medical literature so far.

A retrospective clinical study was performed to evaluate the etiology, diagnosis, and management of gastrointestinal tract perforation (GITP) due to blunt abdominal trauma (BAT) in order to find a predictor to avoid delay in diagnosis. Thirty-five children with GITP out of 805 BAT victims (4.3%) over a 21-year period formed the study group. Different parameters including preoperative (mechanism of injury, abdominal and X- ray findings at presentation, diagnostic modalities), operative (type and site of GITP, intra-abdominal associated injuries, surgical method), and postoperative (complications, mortality) status were analyzed. The patients were subdivided according to their initial clinical presentation as group I: evidence of peritonitis (n = 19, 54%); group II: abdominal findings such as distension, minimal tenderness, and guarding (n = 10, 29%); and group III: normal abdominal findings (n = 6, 26%). These groups were also statistically compared to each other with regard to the parameters mentioned above. The Mann-Whitney U, Wilcoxon rank-sum, and Kruskal-Wallis tests were used for statistical analysis. P

Testicular nodules or tumors have been well described in patients with congenital adrenal hyperplasia (CAH) and usually associated with 21-hydroxylase deficiency. The authors report on a 11-hydroxylase-deficient patient presenting bilateral testicular enlargement and review the literature. Testicular biopsy was not very helpful to make differential diagnosis between adrenal rest hyperplasia and Leydig cell tumor. The size of testes regressed after steroid replacement therapy, and this observation was suggestive for adrenal rest hyperplasia. These findings suggest that bilateral testicular enlargement in patients with CAH may occur after excessive adrenocorticotrophic hormone stimulation of cells differentiated from unknown orgin. Orchiectomy is not required but bilateral testicular biopsy must be performed.

La compression du thorax foetal par la paroi uterine est une des theories proposees pour expliquer le mecanisme de l'hypoplasie pulmonaire associee a l'oligo-amnios. Cependant, la reduction de l'espace intrathoracique et la certitude d'une compression du thorax foetal n'ont jamais ete demontrees en detail. C'est pourquoi une etude experimentale a ete faite pour determiner les effets de l'oligo-amnios sur l'espace intrathoracique utilisable et le volume pulmonaire chez le foetus de lapin. Le fluide amniotique a ete derive dans la cavite peritoneale entre le 23eme et le 31eme jour de gestation. L'espace intrathoracique, le volume pulmonaire (TV et LV) et le poids du poumon et du corps (LW et BW) ont ete mesures et compares avec des foetus a terme. Le rapport poids pulmonaire/poids du corps, le rapport volume pulmonaire/volume du thorax et volume du thorax/poids du corps ont ete calcules et leurs valeurs comparees entre les foetus porteurs d'une derivation et les foetus controles. Les differences entre TV/BW n'etaient pas significatives. LW/ BW et LV/TV ont ete reduites de facon appreciable chez les foetus experimentaux en comparaison des controles. Le drainage chronique du liquide amniotique dans la cavite peritoneale a entraine une reduction de LW/BW et LV/TV mais n'a pas entraine de modification TV/BW. La valeur LV/TV assez basse chez les foetus avec derivation en depit d'un TV/BW identique suggere que le diaphragme s'est trouve releve. C'est aussi une explication de la circonference thoracique reduite qui a ete observee et du rapport circonference thoracique/circonference abdominale dans la rupture prematuree prolongee des membranes chez le foetus humain in vivo. Le poumon foetal doit subir une compression non seulement transthoracique mais egalement trans-abdomino-diaphragmatique. Cependant, cela peut etre different du fait de l'uterus tubulaire du lapin. Dans l'oligo-amnios, la dynamique du fluide amniotique ou ses caracteristiques physiques peuvent etre perturbees. Nos etudes experimentales anterieures concernant l'hypoplasie pulmonaire provoquee par la hernie du sac amniotique entrainant une pression amniotique diminuee sans oligo-amnios suggere egalement le role d'une compression foetale. Quoique le developpement du poumon foetal soit perturbe de facon appreciable, la cage thoracique n'etait pas modifiee par la derivation amnio-peritoneale. En consequence, nous considerons que l'hypoplasie pulmonaire resulte d'un autre mecanisme plutot que de la compression thoracique.

In Hacettepe University Children's Hospital, between 1976 and 1993 two patients among 302 with Hirschsprung's disease were diagnosed with appendiceal perforation (AP) at initial admission. Both patients were less than 2 months of age. One of them was a boy with total colonic aganglionosis and the latter a girl with long-segment disease. In both cases the site of AP was the base, and periappendicitis without mucosal involvement was detected. The present cases and review of the literature suggest that longer aganglionic segment carries a higher risk of AP.

Le role de l'ingestion du liquide amniotique dans le developpement d'organes foetaux differents a ete apprecie chez le lapin. Les foetus de 24 lapins New Zealand blancs ont ete l'objet d'une experimentation au 23eme jour de gestation. Chez ces foetus, une ligature de l'oesophage ou une operation sham est pratiquee. Au 30eme jour de grossesse, une hysterectomie est pratiquee et les diffe-rents systemes organiques des foetus vivants sont examines et leurs organes etudies histologiquement. En meme temps, les enzymes du tractus digestif sont etudies. Il y a 3 morts intra-uterins, 1 chez un : foetus avec une ligature de l'oesophage et 2 chez des lapins operes sham. Le pourcentage de survie est de 85%. Comme prevu, la ligature de l'oesophage amene une augmentation de la quantite de liquide amniotique. On ne trouve pas de difference entre les deux groupes sur le plan du poids du corps foetal ou en ce qui concerne le poids propre des poumons, du coeur, du foie et des reins, de meme que la relation entre le foie et les poumons et le poids du corps. En revanche, le poids de l'estomac, de l'intestin grele, c'est a dire de tout le tractus gastro-intestinal apparait plus reduit chez les animaux ayant subi une ligature oesophagienne que chez les operes sham (p < 0,05). Egalement, on a constate une difference entre le poids de l'estomac et le poids total du corps, de meme pour l'intestin grele et tout le tube digestif, de meme le poids des reins par rapport au poids total du corps est tres reduit (p

The association of appendicitis and blunt abdominal trauma (BAT) is an exceptionally rare occurrence with a few case reports in the literature. The main question whether this association is a result of causative or coincidental relationship has not been evaluated among children. A retrospective clinical study was performed to clarify the pathogenesis, incidence, clinical characteristics and outcome of appendicitis diagnosed in children during the hospitalization period following blunt abdominal trauma (BAT). The records of 554 patients with BAT who were admitted to our unit between 1979 and 1993 inclusive were reviewed. Five patients (0.90%), 3 males and 2 females, with a mean age of 9.4 +/- 1.4 were found to have appendicitis. None of these patients had any signs or symptoms related to appendicitis prior to BAT. Bruising and rupture of the mesoappendix were noted in 3 patients while edema and hematoma of appendix and terminal ileum were found in two. Full recovery was achieved in all patients following appendicectomy. Our incidence of 0.90% is statistically significantly higher than the highest incidence rate of appendicitis stated in the literature during childhood (t-test for population proportion, t = 2.892; p < 0.05). We emphasise the possibility of a causative relationship between appendicitis and BAT depending on the clinical characteristics, operative findings, statistically significant high incidence rate and literature review.

Les pathologies renales bilaterales comme l'agenesie renale et la dysplasie de meme que les obstructions du tractus urinaire inferieur, ont ete rapportees comme cause de l'hypoplasie pulmonaire. L'oligohydramnios et la compression thoracique ont ete proposes comme cause d'hypoplasie pulmonaire. Le mecanisme exact reste totalement inconnu. En plus, les effets de l'absence de tissu renal sur le developpement du foetus n'ont pas ete etudies jusqu'ici en detail. En consequence une etude experimentale a ete planifiee pour preciser les effets de la nephrectomie foetale sur le developpement. Les foetus de 27 lapins blancs New Zealand ont ete etudies au 23eme jour de gestation. Les foetus de l'ovaire droit ont subi une nephrectomie bilaterale ou une operation simulee. Les lapins ont subi une hysterectomie au 30eme jour de gestation et les foetus vivants ont ete etudies. Le poids du foetus du poumon, du coeur et du foie de meme que les volumes du poumon, du coeur et du thorax ont ete precises et le rapport poids des organes/poids du corps a ete precise. De plus, les poumons ont ete etudies histologiquement. Quoique la nephrectomie foetale entraine une diminution du poids du corps (BW), la proportion BW/poids du viscere n'etait pas modifiee en ce qui conceme le coeur, le poumon et le foie. Cependant, les volumes du coeur et du thorax ont ete diminues significativement. En plus, les volumes du coeur et du thorax ont ete diminues significativement dans le groupe nephrectomise (p

An experimental study was performed to investigate the excretion function of the liver, gastrointestinal motility, and in utero defecation by radionuclide techniques in 24 New Zealand white rabbit fetuses at 25 days' gestation (fullterm, 31 to 32 days). 0.1 mL of technetium 99m (99mTc)-HIDA (a derivative of iminodiacetic acid) containing 1 mCi of radioactivity was injected into the gluteus muscle of each fetus which had been exposed through the uterus. After replacing the fetus and uterus into the abdomen, and beginning 1 hour after injection, a live fetus was killed each hour for 24 hours Tissue samples from the lung heart, stomach, kidney, bladder, liver, meconium in the proximal, mid and distal bowel, and amniotic fluid were taken. The radioactivity of each sample was determined by a gamma counter and the percentage uptake per gram of tissue was calculated. The very low radioactivity levels detected in the stomach, kidneys, and bladder indicated the in vivo stability of 99mTc-HIDA 99mTc-HIDA is predominantly trapped by the liver via systemic circulation and is excreted into the gastrointestinal tract through which it passes into the amniotic fluid. Demonstrated passage of excreted 99mTc-HIDA through the fetal liver and into the gastrointestinal tract and amniotic fluid strongly suggests that fetal defecation is a physiological event.

Among 16 with pyogenic liver abscess, two were found to have Papillon-Lefevre syndrome. The two cases are reported herein. Bacteremia from involved periodontal tissues and a possible impaired immune response could indicate an increased risk of pyogenic liver abscess among children with Papillon-Lefevre syndrome.

Nonlymphoid gastrointestinal malignancies are not frequently encountered malignant tumors of childhood. From 1972 to 1994, at Hacettepe University Children's Hospital, there were 6,774 malignant tumors encountered in patients from birth to age 17. Thirty-five of them (0.5%) originated from the gastrointestinal tract. There were five carcinoid tumors, four pancreas tumors (two were solid-cystic tumors), four APUD-omas, three leiomyosarcomas, one mesothelioma, two carcinomas of the stomach and duodenum, and 16 colon and rectum carcinomas (12 were mucin-producing). These rare tumors are discussed according to the characteristics of the patients, therapy modalities applied, and outcome of the patients. (C) 1996 Wiley-Liss, Inc.

The diagnosis of Munchausen syndrome by proxy is frequently difficult to document and often delayed. A unique presentation of a case of Munchausen syndrome by proxy involving insertion of factitious stones in the lower urinary tract is reported.

OBJECTIVE: Our purpose was to examine fetal lung development in reduced intraamniotic pressure without amniotic fluid loss. STUDY DESIGN : A circular portion of uterine wall measuring 1 cm in diameter was excised while the chorionic and amniotic membranes were left intact at the twenty-third day of gestation in Nez Zealand White rabbits. The chorionic and amniotic membranes herniated spontaneously through the defect. RESULTS: Amniotic pressure was significantly reduced after herniation. Lung weight/body weight ratios at term who significantly reduced in experimental fetuses compared with controls. Residual amniotic fluid volumes at term did not differ. Histopathologic examination of lung specimens showed that fetal lungs had not matured in the experimental group as fully as in the control group. CONCLUSION: This experimental study demonstrated that low amniotic pressure impaired fetal lung development, even without oligohydramnios.

Familial Mediterranean fever (FMF) is a disease characterized by recurring and self-limiting attacks of febrile serosal inflammation involving the peritoneal, synovial, and pleural membranes. Peritonitis is the most common clinical picture of FMF, and repeated acute abdominal episodes may result in formation of peritoneal adhesions that may cause adhesive small bowel obstruction (ASBO) requiring surgical intervention. This subject has neither been clarified nor thoroughly evaluated in the literature. The records of 355 pediatric patients diagnosed to have FMF were reviewed in order to clarify the incidence and outcome of ASBO without prior laparotomy during the course of FMF. The incidence rate has been found as 3% with no mortality. This figure shows ASBO to be the most frequent complication of FMF. Therefore this life-threatening surgical emergency should be kept in mind in the differential diagnosis of acute abdominal attacks during the course of FMF.

The effects of chemical sympathectomy on contralateral testicular histology, fertility and fecundity following unilateral testicular torsion were evaluated in rats. Four groups, placebo plus sham operation, 6-OH-dopamine plus sham operation, placebo plus torsion, and 6-OH-dopamine plus torsion, were established. When the placebo plus sham operation and placebo plus torsion groups were compared, it was found that contralateral testicular damage following unilateral testicular torsion occurred with significantly decreased values for mean seminiferous tubular diameter (MSTD), mean testicular biopsy score (MTBS) and fertility. The relatively normal values for MSTD, MTBS and fertility in the 6-OH-dopamine plus sham operation and 6-OH-dopamine plus torsion groups indicate the preventive role of chemical sympathectomy on contralateral testicular damage. Since chemical sympathectomy prevents contralateral histologic deterioration and preserves fertility in unilateral testicular torsion, the decreased blood flow in the reflex-activating sympathetic system may play a role in contralateral testicular damage.

WOS: 000180231400022, PubMed: 12495967, We report a case of infiltrating angiolipoma who presented with a large mass on the shoulder and the signs of Kasabach-Merritt syndrome. She improved dramatically within one month and the mass completely disappeared after six months of interferon treatment.

An in-utero experimental study was performed to evaluate the effects of intrauterine vascular compromise on further development of corpus spongiosum and male urethra. Thirty time-mated pregnant New Zealand white rabbits on their twenty-third day of gestation were used. Deterioration of the blood supply of the corpus spongiosum and urethra was attempted by electrocauterizing the perineum adjacent to the root of the phallus without damaging the corpus spongiosum and urethra, under the operating microscope. A bipolar cautery was carried out using a specially designed forceps having 100 microns interspaced fixed tips. Five experimental and seven control live male litters were delivered at term by Cesarean sections. Their anogenital regions were examined histopathologically. While the corpus spongiosum extended as long as the corpus cavernosum to the tip of phallus in control litters, the corpus spongiosum ended under the skin in one of the experimental group and in the other four was shorter than the corpus cavernosum. In the latter group, no histopathological evidence of tissue destruction which might be related to electrocauterization, was found. Similar to the pathogenesis encountered in intestinal atresia, a vascular insult which might occur even after the completion of organogenesis, may affect the fate of the corpus spongiosum and urethra. Localized ischemia resulting from local vascular insults may explain, at least in theory, the pathogenesis of some congenital anomalies of corpus spongiosum and urethra such as hypospadias.