Gastric emptying of water in children with severe functional fecal retention.

The objective of this study was to evaluate gastric emptying (GE) in pediatric patients with functional constipation. GE delay has been reported in adults with functional constipation. Gastric emptying studies were performed in 22 children with chronic constipation, fecal retention and fecal incontinence, while presenting fecal retention and after resuming regular bowel movements. Patients (18 boys, median age: 10 years; range: 7.2 to 12.7 years) were evaluated in a tertiary pediatric gastroenterology clinic. Gastric half-emptying time of water (reference range: 12 ± 3 min) was measured using a radionuclide technique immediately after first patient evaluation, when they presented fecal impaction (GE1), and when they achieved regular bowel movements (GE2), 12 ± 5 weeks after GE1. At study admission, 21 patients had reported dyspeptic symptoms, which were completely relieved after resuming regular bowel movements. Medians (and interquartile ranges) for GE1 and GE2 were not significantly different [27.0 (16) and 27.5 (21) min, respectively (P = 0.10)]. Delayed GE seems to be a common feature among children with chronic constipation and fecal retention. Resuming satisfactory bowel function and improvement in dyspeptic symptoms did not result in normalization of GE data.

Gastric emptying of water in children with severe functional fecal retention

The objective of this study was to evaluate gastric emptying (GE) in pediatric patients with functional constipation. GE delay has been reported in adults with functional constipation. Gastric emptying studies were performed in 22 children with chronic constipation, fecal retention and fecal incontinence, while presenting fecal retention and after resuming regular bowel movements. Patients (18 boys, median age: 10 years; range: 7.2 to 12.7 years) were evaluated in a tertiary pediatric gastroenterology clinic. Gastric half-emptying time of water (reference range: 12 ± 3 min) was measured using a radionuclide technique immediately after first patient evaluation, when they presented fecal impaction (GE1), and when they achieved regular bowel movements (GE2), 12 ± 5 weeks after GE1. At study admission, 21 patients had reported dyspeptic symptoms, which were completely relieved after resuming regular bowel movements. Medians (and interquartile ranges) for GE1 and GE2 were not significantly different [27.0 (16) and 27.5 (21) min, respectively (P = 0.10)]. Delayed GE seems to be a common feature among children with chronic constipation and fecal retention. Resuming satisfactory bowel function and improvement in dyspeptic symptoms did not result in normalization of GE data.

A functional study of caustic strictures of the esophagus in children.

The objective of the present study was to assess esophageal motor function in 21 children (7.5 +/- 2.9 years) with caustic strictures. Esophageal manometry was performed using a water-infusion system interfaced with a polygraph and displayed on a computer screen. The data were compared with those obtained from 9 healthy children. Radionuclide transit was determined by studying deglutition of a single bolus of 99mTc pertechnetate in 10 ml of water. Non-peristaltic low-amplitude and long-duration waves were the most common findings detected in patients with strictures longer than 20% of esophageal length (N = 11). Compared with the control group, these patients presented lower mean amplitude and longer mean duration of waves (24.4 +/- 11.2 vs 97.9 +/- 23.7 mmHg, P < 0.05, and 6.7 +/- 2.4 vs 1.6 +/- 0.1 s, P < 0.05, respectively). Six patients presented low-amplitude waves just below the constricted site. Ten children presented delayed esophageal transit. There was an association between dysphagia and abnormalities on manometry (P = 0.02) and between symptoms and scintigraphy data (P = 0.01). Dysphagia in caustic strictures is due to esophageal motility abnormalities, which are closely related to the scarred segment.

A functional study of caustic strictures of the esophagus in children

The objective of the present study was to assess esophageal motor function in 21 children (7.5 ± 2.9 years) with caustic strictures. Esophageal manometry was performed using a water-infusion system interfaced with a polygraph and displayed on a computer screen. The data were compared with those obtained from 9 healthy children. Radionuclide transit was determined by studying deglutition of a single bolus of 99mTc pertechnetate in 10 ml of water. Non-peristaltic low-amplitude and long-duration waves were the most common findings detected in patients with strictures longer than 20 percent of esophageal length (N = 11). Compared with the control group, these patients presented lower mean amplitude and longer mean duration of waves (24.4 ± 11.2 vs 97.9 ± 23.7 mmHg, P < 0.05, and 6.7 ± 2.4 vs 1.6 ± 0.1 s, P < 0.05, respectively). Six patients presented low-amplitude waves just below the constricted site. Ten children presented delayed esophageal transit. There was an association between dysphagia and abnormalities on manometry (P = 0.02) and between symptoms and scintigraphy data (P = 0.01). Dysphagia in caustic strictures is due to esophageal motility abnormalities, which are closely related to the scarred segment.

The role of videolaparoscopy in the diagnostic and therapeutic approach of nonpalpable testis

OBJECTIVE: Evaluate the results from the first 5 years of experience with laparoscopy for diagnosis and treatment of nonpalpable testes. MATERIALS AND METHODS: Medical records of 51 patients submitted to laparoscopic testicular exploration, during a 5-year period, were retrospectively analyzed. Patients' mean age was 65.7 months (median = 48) on the first procedure. The youngest patient was 10 months and the oldest was 14 years old on the first surgery. Twenty-four (47 percent) patients presented nonpalpable testes bilaterally, 7 (14 percent) only at the right side and 20 (39 percent) at the left, totaling 75 testicular units assessed. Patients who had their testes palpated after anesthetic induction were excluded from the study, and in all other cases, surgical management was based on the testicular position and viability. During the post-operative follow-up, surgical success was classified as palpable testis in scrotal sac, with adequate consistency and volume. RESULTS: Nine (12 percent) testes were not localized, but their vessels and deferent duct were atrophic. Two (3 percent) testes were intra-abdominal and atrophic, and 2 (3 percent) gonads, in the same patient, had a dysmorphic aspect. Nineteen (25 percent) testicular units were located close to the internal inguinal ring (peeping testes) and, in 22 (29 percent) units, the spermatic vessels and deferent duct penetrated the internal inguinal ring. Eight (10 percent) testes were located at a distance of less than 2 cm from the internal inguinal ring and 13 (17 percent) at a distance greater than 2 cm. The 2 intra-abdominal atrophic testes were removed. Inguinotomy was performed in a total of 41 (54 percent) cases, reaching a surgical success of 89 percent. Laparoscopic orchiopexy in one stage, without vascular ligation, was performed in 9 (12 percent) testes, which presented a distance of less than 2 cm from the internal inguinal ring, also with a surgical success index of 89 percent. Orchiopexy in 2 stages, with ligation of the spermatic vessels, was performed in 13 (17 percent) testicular units located at a distance greater than 2 cm from the internal inguinal ring, reaching 77 percent of good results. CONCLUSION: Videolaparoscopy is a safe and effective method for diagnosis and treatment of nonpalpable testis.

The role of videolaparoscopy in the diagnostic and therapeutic approach of nonpalpable testis.

OBJECTIVE: Evaluate the results from the first 5 years of experience with laparoscopy for diagnosis and treatment of nonpalpable testes. MATERIALS AND METHODS: Medical records of 51 patients submitted to laparoscopic testicular exploration, during a 5-year period, were retrospectively analyzed. Patients' mean age was 65.7 months (median = 48) on the first procedure. The youngest patient was 10 months and the oldest was 14 years old on the first surgery. Twenty-four (47%) patients presented nonpalpable testes bilaterally, 7 (14%) only at the right side and 20 (39%) at the left, totaling 75 testicular units assessed. Patients who had their testes palpated after anesthetic induction were excluded from the study, and in all other cases, surgical management was based on the testicular position and viability. During the post-operative follow-up, surgical success was classified as palpable testis in scrotal sac, with adequate consistency and volume. RESULTS: Nine (12%) testes were not localized, but their vessels and deferent duct were atrophic. Two (3%) testes were intra-abdominal and atrophic, and 2 (3%) gonads, in the same patient, had a dysmorphic aspect. Nineteen (25%) testicular units were located close to the internal inguinal ring (peeping testes) and, in 22 (29%) units, the spermatic vessels and deferent duct penetrated the internal inguinal ring. Eight (10%) testes were located at a distance of less than 2 cm from the internal inguinal ring and 13 (17%) at a distance greater than 2 cm. The 2 intra-abdominal atrophic testes were removed. Inguinotomy was performed in a total of 41 (54%) cases, reaching a surgical success of 89%. Laparoscopic orchiopexy in one stage, without vascular ligation, was performed in 9 (12%) testes, which presented a distance of less than 2 cm from the internal inguinal ring, also with a surgical success index of 89%. Orchiopexy in 2 stages, with ligation of the spermatic vessels, was performed in 13 (17%) testicular units located at a distance greater than 2 cm from the internal inguinal ring, reaching 77% of good results. CONCLUSION: Videolaparoscopy is a safe and effective method for diagnosis and treatment of nonpalpable testis.

Morphometry and histology of gonads from 13 children with dysgenetic male pseudohermaphroditism.

BACKGROUND: Dysgenetic male pseudohermaphroditism (DMP) is a sexual differentiation disorder characterized by bilateral dysgenetic testes, persistent müllerian structures, and cryptorchidism in individuals with a 46,XY karyotype. However, the histologic criteria for the diagnosis of DMP are poorly established. OBJECTIVE: To determine gonadal histology in children with DMP. PATIENTS AND METHODS: Between 1996 and 1998, 13 patients with DMP were evaluated on our service. The clinical diagnosis of DMP was based on a 46,XY karyotype, sex ambiguity, high levels of follicle-stimulating hormone and low levels of antimüllerian hormone, a decreased testosterone response to human chorionic gonadotropin stimulation without accumulation of testosterone precursors, and the presence of müllerian structures. Molecular sequencing the HMGbox region of the SRY gene did not reveal any mutations. Biopsies were performed for 22 of 26 gonads (patient age at the time of biopsy, 16 months to 10 years). Conventional microscopy was used to evaluate mean tubular diameter, tubular fertility index, and number of Sertoli cells per tubular profile. RESULTS: All 26 gonads were located outside of the labioscrotal folds. Their histologic features varied from only a reduction in tubular size to features of a streak gonad. Five of the 22 gonads grossly resembled a streak gonad. The mean tubular diameter was severely reduced (>30% reduction relative to the normal tubular diameter for the patient's age) in 4 gonads, markedly reduced (10%-30%) in 11 gonads, slightly reduced (<10%) in one gonad, and normal in one gonad. The tubular fertililty index, expressed as the percentage of tubular profiles containing germ cells, was severely reduced (<30% of normal values) in 9 gonads, markedly reduced (50%-30%) in 2 gonads, and normal in 6 gonads. The number of Sertoli cells per tubular profile was elevated in 16 gonads and normal in one gonad. Thin tubules surrounded by fibrous tissue were occasionally observed. CONCLUSION: The histologic findings confirmed the clinical diagnosis of DMP in every patient in the present series. However, gonadal histology was variable, and careful morphometric evaluation may be necessary to establish the diagnosis.

Lateral esophagostomy: an alternative in the initial management of long gap esophageal atresia without fistula.

The authors report an alternative method of cervical esophagostomy that was used in a child with type A esophageal atresia. This method involved performing a lateral esophagostomy in the proximal pouch, preserving its distal end, allowing the child to swallow normally, without choking, while stimulating the spontaneous growth of the proximal esophagus. As a result, the infant could be discharged home on G-tube feedings while waiting for spontaneous growth of the proximal pouch to occur. There were no episodes of aspiration during this period, and definitive reconstruction through end-to-end esophageal anastomosis was accomplished successfully at the age of 18 months. The authors consider that this alternative might increase the possibility of a definitive correction through delayed primary anastomosis of the infant's own esophagus in children with this type of malformation.

Selective surgical indication in the management of neutropenic children presenting with acute abdomen.

As the treatment of pediatric malignancies improves and survival increases, the diagnosis of acute abdomen in these patients also becomes more common. Nevertheless, the management of this condition is still controversial. The authors report their experience in treating 12 neutropenic children with acute abdomen. The charts of 12 neutropenic patients with a diagnosis of acute abdomen treated at Boldrini Children's Cancer Center in Campinas, Brazil, between 1991 and 1996, were reviewed. Therapeutic strategy included an initial period of bowel rest, general supportive measures, and broad-spectrum antibiotics while waiting for the neutrophil count to rise. Three patients recovered completely without surgery, 8 underwent late surgery without complications, and 1 died due to uncontrolled sepsis before surgery. The treatment of acute abdomen in neutropenic children remains controversial. As shown in the present series, an initial nonoperative approach with selective surgical indication appears to be safe and to yield good results. Supportive treatment, until the neutrophil count rises, followed by surgery, if necessary, appears to be a sound therapeutic approach for neutropenic children with acute abdomen.

Beckwith-Wiedemann syndrome and virilizing cortical adrenal tumor in a child.

The authors report a case of a virilizing adrenal tumor that developed in a 2-year-old child with Beckwith-Wiedemann syndrome (BWS). He had a fetal diagnosis of omphalocele and a history of neonatal adrenal cysts. The importance of prenatal diagnosis of BWS and postnatal follow-up of tumors is discussed. The differential diagnosis of adrenal pathologies occurring in BWS also is reviewed.

Pre-operative control of arterial hypertension using ketoconazole in pediatric patients with adrenocortical tumors.

Adrenocortical tumors are rare in childhood, appearing more frequently in some regions such as South and South-eastern regions of Brazil and India. Common clinical signs include virilization, Cushing's syndrome, feminization and hypertension, either isolated or in association. The aim of this report is to present our experience with the pre-operative use of ketoconazole in children with an adrenocortical tumor to control elevated blood pressure levels non-responsive to the usual treatment. Over the last 16 years, of 46 children diagnosed as having adrenocortical tumor, 17 developed hypertension (diastolic pressure greater than the 95th percentile for age and sex according to data from the Task Force on Blood Pressure Control in Children), associated with virilization and/or Cushing's syndrome. In three of these 17 patients, conventional antihypertensive therapy failed, and they were treated with ketoconazole (200-300 mg/day). This resulted in rapid control of the blood pressure. It is concluded that in selected patients, ketoconazole may be useful adjuvant therapy for the palliative control of the arterial hypertension secondary to adrenocortical tumors, without side effects.

[Role of anorectal manometry in the differential diagnosis of chronic constipation in children] / Papel da manometria anorretal no diagnóstico diferencial da constipação em crianças.

OBJECTIVES: 1. To evaluate the role of anorectal manometry in recognizing patients with histological abnormalities of the myenteric plexus as the cause of chronic constipation. 2. To correlate clinical features with the etiology of the constipation.METHODS: The etiology of the constipation was identified retrospectively from the medical records of 57 patients submitted to anorectal manometry. The test was performed in the Pediatric Gastroenterology Laboratory of the University Hospital of the State University of Campinas Medical School. The procedure was performed using a single-balloon system in which a water-perfused pressure transducer was interfaced with a polygraph and displayed on a computer screen. Manometry was performed in patients who, after 6 to 23 months of intense medical treatment using osmotic laxatives and dietary fibres (20 g/d), continued to develop fecal impaction and/or needed evacuatory enema. Regularization of bowel movements during at least 24 months follow-up or the visualization of abnormalities in myenteric plexus in rectal biopsy were used as reference points for the definitive diagnosis.RESULTS: Rectoanal reflex was present in 44 of the 57 patients (77%). Thirteen patients with a negative reflex underwent rectal biopsy which showed abnormalities of the ganglion cells in 10. Subsequent manometry performed on those three patients revealed a normal rectoanal reflex. Sensitivity, specificity, positive and negative predictive values of the manometric test were 1.0, 0.94, 0.77 and 1.0, respectively.CONCLUSIONS: Anorectal manometry is a diagnostic technique with very small possibility of error in differential diagnosis between constipation of a chronic functional nature and that which is secondary to ganglion cell abnormalities.

[Choledochal cyst: a 10-year experience] / Cisto de colédoco: experiência de 10 anos.

BACKGROUND: Choledochal cyst is a rare abnormality usually found in infancy and childhood and mainly in females. Outcome depends on early diagnosis, complete resection of the cyst and proper hepaticoenterostomy.OBJECTIVE: The aim of this paper is to evaluate the outcome of 18 patients operated in ten years. METHODS: Retrospective chart review. RESULTS AND DISCUSSION: Age ranged between 20 days and 13 years (mean 3 years). Fifteen (83%) were female and 03 (17%) male. Signs presented included 15 (83%) with jaundice, 11 (61%) with dark urine, 10(55%) with pale colored stools, 09 (50%) with abdominal pain and palpable mass in only 02(11%). Ultrasonography identified the cyst in 14 (77%) patients. Endoscopic retrograde cholangio-pancreatography demonstrated a long common duct in three of four cases. Anatomical types were: 09 type I, 01 type II, 07 type IV and 01 type V. Except for one patient who had a type V cyst, all patients underwent cyst resection plus Roux-en-Y hepaticojejunostomy. There were two (11%) early postoperative deaths related to deterioration of liver function. Thirteen (86%) of the 15 patients available to follow-up were symptom-free without late complications at a mean period of three years, and two (14%) patients developed chronic pancreatitis.

Extended hiatoplasty--a simple maneuver to lengthen the intraabdominal esophagus during abdominal fundoplication.

AIM: The aim of this paper is to describe a simple technique of extended hiatoplasty to achieve intraabdominal placement of the distal esophagus and evaluate it manometricaly. METHODS: Twelve mongrel dogs were submitted to esophageal manometry using a balloon system and continuous pull-through technique, before and after being submitted to laparotomy and extended hiatoplasty. Parameters measured included length of the HPZ, maximal recorded pressure and maximal end-expiratory pressure. Pre- and post-operative values were compared using the Wilcoxon non-parametric test accepting 0.05 as a level of significance. RESULTS: There was a significant post-operative increase in the length of the HPZ from 48.22 (+/- 4.33) mm to 68.69 (+/- 14.17) mm (p < 0.05). No significant change in the recorded values of maximal pressure or maximal end-expiratory pressure could be detected. The results indicate that an additional portion of the esophagus was exposed to the positive intraabdominal pressure and that this extended hiatoplasty can be used, together with a fundoplication, to increase the efficacy of the gastro-esophageal barrier.

Surgical ligation of patent ductus arteriosus in very low birth weight infants: is it safe?

We evaluated the outcome of a combined medical and surgical treatment of patent ductus arteriosus (PDA) in newborns weighing less than 1500 g. Charts were retrospectively reviewed for 76 newborns with a PDA between 1993 and 1997. Thirteen infants had pre-existing conditions prohibiting the use of indomethacin; eight were managed surgically, five medically. The remaining 63 infants received indomethacin therapy. Thirty-two medical failures occurred, requiring surgical ligation of the PDA. Those requiring surgery had a lower average birth weight (847 versus 997 g) and gestational age (26 versus 28 weeks; P < 0.05). Indomethacin treatment was successful in 27 infants. There were only three operative complications: a small pneumothorax, wound bleeding, and a small aortic tear. All recovered uneventfully and no deaths were attributable to the surgical procedure itself. There was no difference in the incidence of respiratory distress syndrome, duration of intubation, sepsis, neonatal enterocolitis, renal dysfunction, bleeding disorders, or intraventricular hemorrhage among both groups. Surgical ligation of a PDA is associated with a high success rate, a low incidence of complications, and no additional morbidity than indomethacin alone. We propose that surgical ligation should be regarded as a first line therapy for very small premature infants who are at higher risk of medical failure.

Epignathus: report of a case with successful outcome.

Epignathus is an extremely rare form of teratoma that arises from the palate or pharynx in the region of the basisphenoid (Rathke's pouch). This condition is associated with a high mortality rate caused by severe airway obstruction in the neonatal period, thus requiring prenatal planning and prompt surgical treatment after birth. The authors describe a case of a giant epignathus that was successfully resected followed by an uneventful recovery.

PHmetria esofagica abreviada: correlacion de valores del indice de reflujo (IR) en examenes de 8 a 24 hs / Eigth hours pHmetry: correlation of the GE reflux index

Los trazados de 23 pHmetrias esofagicas realizadas entre Enero y Agosto de 1995 fueron analizados retrospectivamente para determinar si un periodo mas corto de evaluacion (8hs) podria producir los indices determinador por la monotorizacion de 24 hs. Fueron analizados los siguientes indices en las primeras 8 hs. de trazado y en el trazado completo de 24 hs: IR; indice de reflujo (por ciento de tiempo con pH menor de 4), AUC; rea bajo la curva de pH<4 y 10 indice oscilatorio (por ciento tiempo con pH entre 3,75 y 4,25). Los valores normales indicados en la literatura para cada uno de estos par metros (IR<4 por ciento AUC<2000 y 10<10 por ciento) fueron utilizados para clasificar los trazados en "normales" y "alterados". El IR se mostro "alterado" en 11 trazados de 8 hs. y en 11 de 24 hs. Hubo un falso positivo y un falso negativo. La sensibilidad y especificidad fueron calculadas respectivamente en 90,9 por ciento y 91,6 por ciento y el valor predictivo positivo en 90,9 por ciento. Los valores de AUC y OI se encontraron "normales" en todos los trazados a las 8 y 24 hs y no fue posible analizarlos. Aunque muchos trabajos afirman que la pHmetria abrevida tiene indices de sensibilidad inferiores a los observados en ex menes de 24 hs, segun nuestros resultados podemos afirmar que el IR tomado en un periodo de 8hs durante el dia es representativo de 24 hs. y se puede utilizar para confirmarel diagnostico de reflujo de la infancia

Effect of fundoplication on the gastric emptying of liquids.

To evaluate the effect of fundoplication on the gastric emptying (GE) of liquids, the authors studied 96 male Wistar rats divided into three main groups: group E (early postoperative), formed by 32 rats that received physiological saline as a test meal and whose gastric emptying was evaluated 8 days after surgery; group L (late postoperative), which received the same test meal but was evaluated 29 days after surgery; and group G (glucose), which received 5% glucose in water and was studied 8 days after surgery. Each group was subdivided in two subgroups of 16 animals: in one (atropine), the animals received intravenous (I.V.) atropine sulfate (0.3 mg/100 mg rat weight) 60 minutes before GE test; the other subgroup (controls) received I.V. physiological saline. In both subgroups 8 animals had been submitted to fundoplication and 8 to sham operation. Every test meal, containing 6 mg% red phenol, was infused by gravity through a metallic catheter. Gastric retention was determined by measuring the concentration of the marker in the liquid recovered from the stomach 10 minutes after infusion. In the animals of group E, fundoplication increased the gastric emptying of physiological saline, both in the control and the atropine subgroups. In the L group, gastric retention values were similar in fundoplication and sham-operated rats, suggesting an adaptation of the stomach to the fundoplication. In the G group, fundoplication enhanced GE among the control animals, but not among those receiving I.V. atropine sulfate. These results support the importance of gastric emptying studies in every patient to be submitted to fundoplication.

[The role of the abdominal ultrasonography and percutaneous liver biopsy in the differential diagnosis of neonatal cholestasis]. / Valor da ultra-sonografia abdominal e da biopsia hepática percutânea no diagnóstico diferencial da colestase neonatal.

Neonatal cholestasis still presents a diagnostic challenge, both from the anatomic and etiologic point of view. Distinguishing intrahepatic from extrahepatic causes of cholestasis is of paramount importance since the latter may be treated by surgery but prognosis depends on the age at which operation is performed. Many tests have been proposed to help in differentiating these two entities, among which liver biopsy is the most frequently employed, and ultrasonography one of the most recently included. Our purpose is to present our experience with these two methods in the differential diagnosis of intra and extrahepatic causes of cholestasis. From January 1989 to July 1993, 35 patients with neonatal cholestasis were evaluated through a protocol which included liver biopsy and ultrasonography. The latter was performed after a 4 hour fast and was considered indicative of extrahepatic cholestasis when the gallbladder was not visualized, was hypoplastic or non-functioning, or if a cystic structure was seen at the extrahepatic biliary tree. Sensitivity, specificity, and accuracy were determined for these two tests. Seventeen patients were found to have extrahepatic cholestasis (all with biliary atresia) and 18 intrahepatic cholestasis, on the basis of clinical evolution or operative findings. Sensitivity was 100% for ultrasonography and 76% for liver biopsy in diagnosing extrahepatic cholestasis. Accuracy was 83% for ultrasonography and 86% for biopsy, rising to 96% when both tests were considered together. Based on these findings we strongly recommend ultrasonography with definite criteria as the initial investigation tool in the management of neonatal cholestasis, associated with liver biopsy.