The use of pH monitoring and esophageal manometry in the evaluation of results of surgical therapy for gastroesophageal reflux disease.

BACKGROUND/PURPOSE: Surgery is indicated for the treatment of gastroesophageal reflux disease (GERD) when medical treatment fails or complications are encountered in children. However, it has not been fully established how the results after surgery can be evaluated. A prospective study was performed to evaluate the results of surgical therapy for GERD by pH monitoring (PM) and esophageal manometry (EM) in children. METHODS: Patients who were candidates for anti-reflux surgery between 2003 and 2004 were evaluated for symptoms, growth and results of PM and EM both in the pre- and postoperative periods. RESULTS: Thirteen patients were included (mean age = 6.65 +/- 3.28 years, male/female ratio = 10/3). Most frequently occurring symptoms were recurrent respiratory infections (RRI) (n = 11) and vomiting (n = 8). Nissen fundoplication was performed because of unresponsiveness to treatment (n = 10), RRI (n = 9), failure to thrive (n = 7) and esophagitis (n = 2) after medical treatment (2 - 36 months). Symptoms were resolved in 83.9 % of patients and were not changed in 16.1 % following surgery. Weight percentiles had significantly improved (pre: 12.38, post: 25.4, p < 0.05) during a short follow-up period (1 - 4 months). Mean reflux index (pre: 24.73 +/- 21.07 %, post: 0.93 %, min: 0 - max: 3.6, p < 0.05), reflux time (pre: 368 +/- 313 min, post: 17.1 +/- 15.9 min, p < 0.05), number of episodes (pre: 344.7 +/- 343.6, post: 19.53 +/- 11.13, p < 0.05) and number of reflux episodes longer than 5 minutes (pre: 4.3, min: 0 - max: 58, post: 0.61, min: 0 - max: 3, p < 0.05) were found to be reduced after surgery by PM. Lower esophageal sphincter pressure (pre: 55 +/- 27 cmH (2)O, post: 64.46 +/- 30.85 cmH (2)O), contraction amplitude (pre: 141.92 +/- 69.11 cmH (2)O, post: 130.69 +/- 45 cmH (2)O) and contraction velocity (pre: 1.94 cm/s, min: 0.1 - max: 7.5, post: 4.29 cm/s, min: 0.2 - max: 10) did not differ postoperatively (p > 0.05). However, contraction times were decreased postoperatively (pre: 73.6 +/- 52.9 s, post: 27.67 +/- 20.1 s, p < 0.05) and were found to be correlated with reflux time and the number of reflux episodes longer than 5 minutes. CONCLUSION: Nissen fundoplication is effective for the treatment of GERD. It supports the anti-reflux mechanism without affecting esophageal motility except for contraction times. The decrease in contraction time after surgery can be explained by the decreases in reflux time and in the number of reflux episodes longer than 5 minutes. PM and EM confirmed the clinical improvement and can be used for the evaluation of results of NF.

Bannayan-riley-ruvalcaba syndrome from the point of view of the pediatric surgeon.

Bannayan-Riley-Ruvalcaba syndrome (BRRS) is an overgrowth syndrome and polyposis condition, which carries an increased risk for development of benign and malignant tumors of various tissues and organs. A 9-year-old boy with BRRS who had multiple subcutaneous mesenchymal tumors and follicular adenoma of the thyroid and a 14-year-old boy with multiple subcutaneous mesenchymal tumors and gastrointestinal polyposis are presented, with special emphasis on the surgical management of these patients.

Sigmoid colon vaginoplasty in children.

BACKGROUND: Vaginal construction is necessary for the patients with aplasia of Mullerian ducts, testicular feminisation and androgen insensitivity syndromes. Many methods of vaginal construction have been described. We report here the outcomes of six adolescent patients who underwent sigmoid colon vaginoplasty with special emphasis on the surgical technique and outcomes. PATIENTS AND METHODS: Between 1990 and 2003, six patients underwent sigmoid vaginoplasty after a diagnosis of 5alpha-reductase deficiency (n = 3), testicular feminisation (n = 2) or vaginal atresia (n = 1). The mean age was 16 years (13 to 18). Wide spectrum antibiotics and whole-gut preparation were used in all cases. A 15-20 cm segment of sigmoid colon was pulled through the retrovesical tunnel. The proximal end was closed in two layers in patients with 5alpha-reductase deficiency and with testicular feminisation. A distal anastomosis was carried out to the opening made on the vaginal plate (5alpha-reductase deficiency) or on the tip of the shallow rudimentary vagina (testicular feminisation). The sigmoid segment was interposed between the blind end of the atretic vagina and the perineum in the patient with vaginal atresia. Patients were instructed to perform daily vaginal irrigation. The neovagina was examined and calibrated under anaesthesia. No routine vaginal dilatation was recommended. RESULTS: All but one patient had an uneventful postoperative period and were discharged within 7-8 days. All patients had an excellent cosmetic result with an appropriate vaginal length. One of the patients experienced late stenosis of the introitus which responded to dilatations. Mucus discharge was not a significant problem. The patient with vaginal atresia (Bardet-Biedl syndrome) experienced deep vein thrombosis, renal failure and sepsis, resulting in death. CONCLUSION: Sigmoid colon vaginoplasty is a special procedure which appears appropriate for the construction of a new vagina in children. A sigmoid colon neovagina meets all necessary criteria after a vaginoplasty. It provides an adequate diameter and length, and produces less scar tissue in the perineum. It is self-moistening, easily adaptable to the uterus, cervix and rudimentary atretic vaginal segments and does not require routine dilatation. Mild stenosis of the introitus can be treated by dilatations and revision can be easily performed in severely stenotic cases. On the other hand, the patient may face morbidity after laparotomy and other serious complications may occur due to accompanying diseases.

Management of bilateral Wilms tumor over three decades: The perspective of a single center.

INTRODUCTION: Outcomes of Wilms tumor improved in last 50 years and excellent survival rates can be achieved especially in case of non-metastatic disease and favorable histology. Nevertheless, bilateral cases still stand as a therapeutic challenge. Prognosis of bilateral Wilms tumor (BWT) is not as good as the unilateral tumors of similar stage and histology in terms of survival and renal function. OBJECTIVE: Management of BWT is constantly evolving and still stands as a therapeutic challenge. This study is designed to review and share our experiences on this topic from a surgical standpoint. STUDY DESIGN: The records of patients treated in our clinic between 1980 and 2013 according to Turkish Pediatric Group of Oncology protocol were analyzed retrospectively and clinical data, surgical details, pathology results, long term outcomes were analyzed. RESULTS: Thirteen girls and 7 boys with a mean age of 2,5 years were treated. There were 2 patients with Wilms tumor-Aniridia-Growth Retardation complex and one with isolated hemihypertrophy. Metastasis were detected in lungs of 4 patients; liver of 2 and in the cranium of one. All patients except one with the presumptive diagnosis of unilateral Wilms tumor were given preoperative chemotherapy. To sum up; 19 nephroureterectomies, 8 partial nephrectomies and 13 enucleations were performed to 36 kidneys without any major early or late postoperative complications. Pathologic results revealed positive surgical margins in 2 lesions with enucleation and in 2 with partial nephrectomies and anaplasia in 4 patients. Two patients were not operated due to parental disapproval. Two patients had the need of dialysis; one was anephric and the others' renal functions recovered over a year. Seven patients received radiotherapy for pulmonary metastasis, positive surgical margins or local recurrences. Overall, 13 patients survived and 7 died due to metastasis, recurrences, and complication of dialysis and refusal of surgical treatment. Survival among all patients was 65% and 72.2% among operated ones. Of the 7 patients with the partial nephrectomy, 2 died and 5 survived. Among enucleation group, 8 out of 10 survived and 2 died. Survival was slightly higher among enucleation group (80% vs 71.4%). Median time of follow-up for survivors of disease is 5.8 years (min: 6 months and max: 14 years). DISCUSSION: Outcomes of BWT management have changed dramatically during the last few decades from only survival, to a long life expectancy without the need of renal replacement therapy owing to improvements in treatment options. We argue that positive surgical margins do not necessarily lead to local recurrence. For this reason it may be wiser to favor on more nephron sparing surgery than to achieve negative surgical margins. Adjuvant chemotherapy and radiotherapy may be adequate to prevent local recurrence. Also, survival did not differ significantly between different ways of nephron sparing surgeries, so it may be wiser to choose enucleation over partial nephrectomy which preserves more nephrons. Nephron-sparing surgery should have utmost importance despite the risk of positive margins. On the other hand, there is not enough data to interpret if positive surgical margins have role on distant metastases or not. Presence of metastasis and recurrence seems to be an important determinant of prognosis given the fact that none of the survivors had any metastasis or recurrence. CONCLUSION: Nephron preservation should be the aim while taking positive surgical margin risk on nephron sparing surgery side relying on postoperative chemotherapy and carefully planned radiotherapy to avoid recurrence. However, there is significant diversity on the management BWT in different centers and a certain validated guideline or protocol to provide the optimal treatment is still lacking.

Bilateral Poland anomaly.

Bilateral absence of the pectoralis major muscle with accompanying abnormalities of shoulder muscles has been reported in patients without Poland anomaly (PA). However, symmetric absence of pectoralis major muscles, hypoplasia of breasts and nipples with symmetric chest wall deformity and bilateral hand anomaly has not previously been reported. A 6-year-old girl with bilateral absence of pectoralis major muscles and hand involvement and symmetric chest wall deformity is, to our knowledge, the first known case of bilateral Poland anomaly.

Late presentation of Bochdalek hernia: clinical and radiological aspects.

Three infants with late presentation of Bochdalek hernia are presented. The presenting symptoms were cough, intermittent vomiting, dyspnea, and cyanosis. Initial diagnoses of isolated paravertebral mass and foreign material aspiration were made in two infants, based on plain chest x-ray findings and history of the patients. Further radiological investigations, such as contrast upper gastrointestinal series or enema, computerized tomography, and magnetic resonance imaging of the chest, suggested the diagnosis of Bochdalek hernia. The hernia was found on the left side in two patients and on the right side in one. At operation, the stomach, small intestine, and spleen were found as herniated organs in one patient, ascending colon in one, and all of the small intestine together with ascending colon in the other. A congenital diaphragmatic defect should be suspected in every child presenting with unusual respiratory or gastrointestinal symptoms and with abnormal chest x-ray findings. The radiological findings vary greatly from one case to another, and even in the same case at different times because of differences in herniated organs and intermittent spontaneous reduction. The possibility of congenital diaphragmatic hernia should be kept in mind to avoid a wrong diagnosis, undue delay in diagnosis, and inappropriate treatment.

Congenital urethrocutaneous fistula: a case report and literature review, with a nomenclature proposal.

The authors report the case of a 4-year-old boy who had a congenital urethrocutaneous fistula (CUF) on the penile urethra. The previous reports of patients with fistula from the urethra to the skin are evaluated, and the spectrum of CUF is discussed.

Adrenal calcification in children: a case report.

Unilateral adrenal calcification was encountered incidentally in a 9-year-old boy during evaluation of enuresis. He was a large baby at birth, and perinatal history was suggestive of difficult labor. The case has been discussed through the possible causes of adrenal calcification and the management of these children. Once the diagnosis of idiopathic adrenal calcification is made in light of detailed perinatal history, biochemical investigations, and roentgenologic examinations, these children can be observed by control evaluations with regular intervals.

Hydatid cyst: an unusual etiology for a cystic lesion of the posterior mediastinum.

The cystic lesions of the posterior mediastinum include bronchogenic, enteric and intramural esophageal cysts, lymphangioma, and anterior meningocele. Hydatid cysts are usually located in the liver, lung, and brain. Although many uncommon locations have been reported, hydatid cyst has not previously been encountered as an isolated posterior mediastinal lesion. A 4-year-old boy with a posterior mediastinal cystic lesion, which has been proven to be a hydatid cyst, is reported.

In utero defecation of the nondistressed fetus: a roentgen study in the goat.

An experimental study was performed to investigate gastrointestinal motility and meconium passage, with simultaneous blood gas measurements, in the fetuses of eight pregnant goats at 110 to 114 days' gestation (full term, 147 to 155 days). With the goats under halothane anesthesia, a nasogastric tube and a heparinized central venous catheter were inserted into the fetuses. 24 hours after surgery, 10 mL of gastric juice from the fetus was replaced with a nonhydrosoluble contrast medium, and serial roentgenograms and blood samples (for pH, PO2, and PCO2 measurement) were taken every 4 hours. All fetuses began to pass the contrast medium into the amniotic cavity within 16 to 22 hours, and central venous blood gas values were normal. The results of this study suggest that the fetus, which urinates routinely, also defecates routinely into the amniotic cavity, even in the absence of distress. The increased incidence of meconium staining in fetal distress conditions may be indicative of impaired clearance of amniotic fluid.

Spontaneous rupture of choledochal cyst: an unusual cause of acute abdomen in children.

Rupture is one of the rare complications of choledochal cysts. The cause of rupture is unknown in many cases and is considered to be spontaneous. Two patients admitted to the hospital with acute abdominal picture were initially thought to have postoperative abscess and appendicitis. However, the presence of bilelike fluid suggested a bile tract pathology, and evaluation showed rupture choledochal cysts. Although spontaneous rupture of a choledochal cyst is a rare complication, and can sometimes be the initial manifestation of a choledochal cyst, it should be considered in the presence of bilelike fluid and differentiated from spontaneous perforation of the bile tract. In spontaneous rupture of choledochal cyst, cystectomy, cholecystectomy, and Roux-Y hepaticoportoenterostomy is the treatment of choice.

Testicular enlargement in patients with 11-hydroxylase deficiency.

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 origin. Orchiectomy is not required but bilateral testicular biopsy must be performed.

Granulocytic sarcoma of the scapula: an unusual presentation of acute myeloblastic leukemia.

The unusual presentation of acute myeloblastic leukemia as a scapular granulocytic sarcoma in an infant without systemic manifestations is reported for the first time. Granulocytic sarcoma as a presentation of leukemia should be considered in the differential diagnosis of scapular masses during childhood. Surgery is limited to obtain sufficient tissue for histopathologic diagnosis.

Enteritis cystica profunda causing ileoileal intussusception in a child.

Enteritis cystica profunda (ECP) is an exceedingly rare lesion of the small intestine. It has identical histopathologic features as colitis cystica profunda, which is mainly a disease of adults and a rare benign lesion of the distal colon and rectum of uncertain cause. Only 12 cases of ECP with only one pediatric case, have been reported in English-language medical literature. Here the authors present the second pediatric case of ECP with the literature review and discussion of the etiology.

Transverse testicular ectopia with persistent müllerian duct syndrome.

Transverse testicular ectopia is rarely associated with persistent müllerian duct syndrome. The ninth pediatric case of transverse testicular ectopia with persistent müllerian duct syndrome is reported. The clinical and operative findings and treatment are discussed. The importance of abdominal exploration in the presence of two gonads in one inguinal side and the avoidance of dissection of müllerian structures has been stressed.

Congenital lobar emphysema: diagnostic and therapeutic considerations.

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 clarify 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.

Colorectal carcinoma in children.

BACKGROUND/PURPOSE: Colorecta I 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.

Granulomatous osteomyelitis of the sternum presenting with a parasternal mass: a possible relation to the bacillus Calmette-Guérin vaccine.

Because they are frequently malignant, swellings of the chest wall during infancy are alarming lesions. Sternal osteomyelitis is a rare condition that may present with a chest wall mass locating in the parasternal region. A parasternal mass of granulomatous osteomyelitic origin is an extremely rare cause with only few case being reported to date. Herein, the authors report on 2 infants presenting with parasternal masses of sternal granulomatous osteomyelitic origin and discuss the possible etiologic role of the bacillus Calmette-Guérin vaccine.

The diagnosis and treatment of H-type tracheoesophageal fistula.

BACKGROUND: H-type tracheoesophageal fistula (TEF) was diagnosed in 12 patients during the 25 years from 1971 to 1996. Although all patients were symptomatic since birth, in 58% of the cases, there was a diagnostic delay ranging from 26 days to 4 years. METHODS: Esophagography and/or cineesophagography, or bronchoscopy and simultaneous methylene blue administration confirmed the diagnosis of H-type TEF. Although cineesophagography is highly effective in demonstrating H-type TEF today, bronchoscopy must be used in every patient suspected of having fistula, especially when the radiological methods fail. Bronchoscopy is helpful in diagnosis, in evaluation of associated respiratory tract anomalies, and in treatment. RESULTS: Two newborn patients could not have been operated on because of pulmonary insufficiency resulting from delayed recognition of fistula. Ten patients underwent fistula repair, nine through cervical approach, and one through thoracotomy, with seven survivors. CONCLUSIONS: The results suggest that early diagnosis is essential in the newborn period. Oversight in identification of H-type TEF, unsatisfactory radiological method, masquerading symptoms related to associated anomalies, and minute symptoms in some patients may cause delay in diagnosis. Radiological and endoscopic procedures are complementary in both diagnosis and treatment of H-type TEF.

In utero defecation by the normal fetus: a radionuclide study in the rabbit.

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.