Optimum therapeutic strategy for meconium-related ileus in very-low-birth-weight infants.

BACKGROUND/PURPOSE: therapeutic strategy for meconium-related ileus (MRI) in very-low-birth-weight infants (VLBWs) has not been established. This study aims to clarify the optimum therapeutic strategy for MRI in VLBWs. METHODS: MRI was defined as delayed meconium excretion and microcolon on contrast enema with Gastrografin (diatrizoate acid). Forty-two infants with MRI were treated at our institution between 2009 and 2019, and are reviewed here. They were classified into two groups: in group A (n=21), Gastrografin regurgitated into the dilated intestine during the first or second round of Gastrografin enema (GaE), while in group B (N = 21), Gastrografin did not regurgitate. Laparotomy was indicated if the intestine was perforated, or if abdominal distention was not relieved by two rounds of GaE. RESULTS: in group A, meconium was excreted in all cases within 24 h after GaE, and no cases required laparotomy. In group B, twelve cases (57%) underwent laparotomy (P < 0.01), six cases in this group (29%), showed free air on X-ray images (P < 0.01). The median hospital stay in groups A and B were 89.0 and 136.5 days, respectively (P < 0.05). Overall mortality was 2.4%. CONCLUSIONS: early therapeutic diagnosis by GaE followed by early surgery is suggested as the optimum strategy for MRI in VLBWs.

Efficacy and safety of strong acid electrolyzed water for peritoneal lavage to prevent surgical site infection in patients with perforated appendicitis.

PURPOSE: Our previous experimental study of perforated peritonitis in rats proved that peritoneal lavage with strong acid electrolyzed water (SAEW) has no adverse effects, reduces the bacteria count in the ascitic fluid more effectively than saline, and increases the survival rate significantly. Thus, we conducted a randomized controlled study, applying SAEW in the treatment of perforated appendicitis in children. METHODS: Forty-four patients, aged 3-14 years, were randomly divided into two groups: Group S (n = 20), in which the peritoneal cavity was lavaged with 100 ml/kg saline and the wound was washed out with 200 ml saline; and Group E (n = 24), in which the peritoneal cavity was lavaged with 100 ml/kg SAEW and the wound was washed out with 200 ml SAEW. RESULTS: No adverse effect of SAEW was observed in Group E. There was no difference in the bacterial evanescence ratio of ascitic fluid after lavage between Groups S and E (11.1 and 15.8%, respectively). A residual abscess developed in one patient from each group (5.0 and 4.2%, respectively). The incidence of surgical site infection (SSI) was significantly lower in Group E than in Group S (0 and 20%, respectively; P < 0.05). There was no difference in the duration of pyrexia, positive C-reactive protein, leukocytosis, or hospital stay between the groups. CONCLUSION: Peritoneal lavage and wound washing with SAEW have no adverse effects and are effective for preventing SSI.

Management of perianal abscess with hainosankyuto in neonates and young infants.

BACKGROUND: Perianal abscess (PA) is a common condition acquired in infancy, yet its treatment method remains controversial. We reviewed the outcome of neonates and young infants with PA who were treated with the traditional Japanese medicine, hainosankyuto (TJ-122). METHODS: Fifteen male infants with PA under the age of 3 months were reviewed. The median age of infants at disease onset was 33 days (range, 18-88 days) and the median bodyweight was 4.1 kg (range, 2.5-6.4 kg). TJ-122 was administered at a dose of 0.20 g/kg/day (n= 13) or 0.25 g/kg/day (n= 2) orally in two or three divided doses before meals. Antibiotics were not used in any of the patients. RESULTS: Of the 15 patients, 14 were cured and had no recurrence, with a median TJ-122 administration of 28 days (range, 14-117 days). Eight patients were cured within 28 days (53%) and 12 were cured within 60 days (80%). One patient, who was later diagnosed with growth hormone deficiency, showed incomplete healing of PA with intermittent pus discharge and recurrence. The patient was cured by 1 year of age following repeated administration of TJ-122 and juzentaihoto (TJ-48). CONCLUSION: Medical management with TJ-122 was effective in most neonates and young infants with PA. It appears prudent to manage these patients with hainosankyuto before resorting to surgical intervention.

Meconium-related ileus in extremely low-birthweight neonates: etiological considerations from histology and radiology.

BACKGROUND: A nationwide survey on neonatal surgery conducted by the Japanese Society of Pediatric Surgeons has demonstrated that the mortality of neonatal intestinal perforation has risen over the past 15 years. The incidence of intestinal perforation in extremely low-birthweight (ELBW) neonates has been increasing as more ELBW neonates survive and as the live-birth rate of ELBW has increased. In contrast to necrotizing enterocolitis (NEC) and focal intestinal perforation (FIP), the pathogenesis of meconium-related ileus, defined as functional bowel obstruction characterized by delayed meconium excretion and microcolon, remains unclarified. METHODS: The histology of 13 ELBW neonates with intestinal perforation secondary to meconium-related ileus was reviewed, and the radiology of 33 cases of meconium-related ileus diagnosed on contrast enema was reviewed. Specimens obtained from 16 ELBW neonates without gastrointestinal disease served as age-matched controls for histological assessment. RESULTS: The size of the ganglion cell nucleus in meconium-related ileus and in control subjects was 47.3 ± 22.0 µm(2) and 37.8 ± 11.6 µm(2), respectively, which was not significantly different. In all cases of meconium-related ileus, contrast enema demonstrated a microcolon or small-sized colon, with a gradual caliber change in the ileum and filling defects due to meconium in the ileum or colon, showing not-identical locations of caliber changes and filling defects. CONCLUSION: Morphological immaturity of ganglia was not suggested to be the pathogenesis of meconium-related ileus. Impaction of inspissated meconium is not the cause of obstruction, but the result of excessive water absorption in the hypoperistaltic bowel before birth, although the underlying mechanism responsible for the fetal hypoperistalsis remains unclear.

Therapeutic strategy for persistent cloaca: the efficacy of antegrade continent enema as a salvage surgery.

PURPOSE: The aim of this study is to evaluate our therapeutic strategy for persistent cloaca from the viewpoint of long-term functional outcome. MATERIALS AND METHODS: This study covers 17 cases of persistent cloaca treated at our institution and followed for more than 3 years. As a definitive repair for anorectal and urogenital systems, simultaneous surgery with posterior sagittal approach or anorecto-urethrovagino-plasty (PSARUVP) was performed. The length of the common channel and the shape of the vagina determined the vaginoplasty methods. Fecal function was assessed with the scoring system of the Japan Study Group of Anorectal Anomalies. RESULTS: Anorectoplasty was performed with the posterior sagittal approach in 15 cases and with the perineal approach in two. Vaginoplasty was performed with total urogenital mobilization in nine cases, rectal interposition in four, vaginal flap in two and with other methods. Fecal function was classified as good in three cases, moderate in ten, and poor in four. In the poor cases, Malone's antegrade continence enema (MACE) was performed, which improved fecal function significantly. CONCLUSION: PSARUVP might be the optimal surgery for persistent cloaca at present; however, satisfactory fecal function could not be achieved in those cases with a longer common channel. MACE effectively compensated for the poor outcome and was especially successful at eliminating incontinence.

Physiological and clinical characteristics of gastroesophageal reflux after congenital diaphragmatic hernia repair.

BACKGROUND/PURPOSE: Gastroesophageal reflux (GER) is an important sequela of congenital diaphragmatic hernia (CDH) repair. This study investigated the physiological and clinical characteristics of GER in CDH survivors. METHODS: A total of 52 CDH survivors were investigated retrospectively. Esophageal acid exposure was evaluated with 24-h esophageal pH monitoring in all patients, and esophageal anatomical and motor functional abnormalities were examined with videomanometry in 16 patients. RESULTS: Fundoplication was necessary in 1 patient. Medical treatment with acid suppression or rikkunshito, a traditional Japanese medicine, was successful in nine patients, and the reflux symptoms were ameliorated at the age of 3 years. The percentage of total time the esophageal pH was below 4.0 (reflux index: RI) ranged from 0.1 to 44.3%. No patient with an RI < 10% had reflux symptoms requiring treatment. The basal lower esophageal sphincter (LES) tone ranged from 15 to 35 mmHg (median 25 mmHg). Esophageal peristalsis was preserved in all of the patients examined, except one who had failed peristalsis and poor clearance in the dilated esophagus. CONCLUSION: The motor function of the esophageal body and LES is usually preserved in CDH survivors despite the wide range of esophageal acid exposure in early infancy. Those with symptomatic GER outgrow it, unless associated with advanced respiratory distress or neurological impairment.

Should fundoplication be added at the time of gastrostomy placement in patients who are neurologically impaired?

BACKGROUND/PURPOSE: Patients who have advanced neurologic impairment (NI) and require gastrostomy placement (GP) frequently have symptomatic gastroesophageal reflux. We investigated the outcomes of GP without fundoplication in patients who had NI. METHODS: This was a retrospective review of 54 patients with NI (median, 7 years; range, 1-18 years) undergoing GP alone. The operative criteria included medically controllable or no reflux symptoms. The patients were divided into 2 groups based on the percentage of total esophageal time with a pH less than 4.0 (reflux index, or RI): group I (GI, n = 33), RI less than 5.0% (median age, 6 years; range, 2-15 years); group II (GII, n = 21), RI 5.0% or greater (median age, 10 years; range, 1-18 years). Data are expressed as medians and ranges. RESULTS: Nutritional management was successfully conducted after GP with or without the administration of lansoprazole, famotidine, or rikkunshito in all but 2 patients. One GI patient with alpha-thalassemia required fundoplication, and one GII patient with Cockayne syndrome required gastrojejunal tube feeding. The RI increased significantly in GI patients (2.1% [0%-4.8%] vs 4.5% [0.2%-11.4%], P = .004), whereas it decreased significantly in GII patients (11.2% [5.9%-41.6%] vs 9.8% [1.05-26.6%], P = .04). CONCLUSION: Gastroesophageal reflux and related symptoms rarely deteriorate to require additional treatment after GP in patients with NI. Gastrostomy placement is a less invasive and effective procedure for improving the quality of life in those patients.

Impact of rikkunshito, an herbal medicine, on delayed gastric emptying in profoundly handicapped patients.

PURPOSE: Rikkunshito is used to treat functional dyspepsia in adults. This study investigated the effects of rikkunshito on delayed gastric emptying in handicapped patients. METHODS: A retrospective review was performed in nine profoundly handicapped patients (aged 1-19 years). All were diagnosed with delayed gastric emptying based on their half gastric emptying time (T(1/2)) over 90 min. Gastric emptying was evaluated after the ingestion of liquid meals using the (13)C-acetate breath test and the BreathID system. Participants were given rikkunshito [0.3 g/(kg day)] with the aim of accelerating gastric emptying. Parameters related to gastric emptying before and during rikkunshito administration were compared using the Wilcoxon signed-rank test. Data were expressed as the median (range). RESULTS: Emesis and hematemesis were relieved with rikkunshito administration in four symptomatic patients. The T(1/2) and T(lag) decreased significantly during rikkunshito administration from 115 min (94-167 min) to 107 min (64-66 min; p = 0.02), and from 60 min (42-90 min) to 47 min (29-59 min; p = 0.03), respectively. The gastric emptying coefficient did not show a significant change [3.1 (2.8-3.8) vs. 3.2 (2.6-4.0), p = 0.15)] with rikkunshito treatment. CONCLUSION: The administration of rikkunshito resulted in symptomatic relief and improved gastric emptying in profoundly handicapped patients with delayed gastric emptying.

Pediatric thyroid cancer arising after treatment for pleuropulmonary blastoma.

A 3-year-old female presented with a large tumor occupying the right thoracic space. Open biopsy revealed the pathological diagnosis of pleuropulmonary blastoma. After the first-line chemotherapy, the patient underwent surgical resection, then two courses of high-dose chemotherapy. Three years later, follicular carcinoma of the right thyroid lobe was found, so a right hemithyroidectomy was performed. Five months later, the thyroid tumor recurred. The remaining thyroid lobe was completely excised and radioiodine therapy was administered. The patient has remained tumor-free for 3 years. The etiology and treatment of the uncommon combination of pleuropulmonary blastoma and thyroid carcinoma is discussed.

Effects of rikkunshito on the clinical symptoms and esophageal acid exposure in children with symptomatic gastroesophageal reflux.

Rikkunshito (TJ-43), a herbal medicine consisting of eight herbs, is used to treat chronic dyspepsia. Studies have shown that TJ-43 improves human gastric emptying. This study investigated the effects of TJ-43 on the clinical symptoms and esophageal acid exposure in children with symptomatic gastroesophageal reflux (GER). Eight children, aged from 2 months to 15 years (median age 4 years), were studied. Six of them had neurological impairment. TJ-43 (0.3 g/kg/day) was given orally or via nasogastric tubes in three divided doses before meals for 7 days. Their symptoms were frequent emesis in four, nausea in two, and hematemesis and stridor in one each. Twenty-four-hour esophageal pH monitoring was conducted using multichannel pH electrodes located at the distal esophagus (P1) and 10 cm proximal to P1 (P2). The clinical symptoms and esophageal pH were compared before and after TJ-43 therapy for 1 week. The frequency of emesis decreased in three patients. Other symptoms, including nausea, hematemesis, and stridor, were relieved in the remaining patients. Measured at the distal pH electrode, the percentage time of esophageal pH < 4.0 and the mean duration of reflux decreased significantly (P < 0.05). However, the number of acid reflux per hour did not change significantly, and no pH parameters measured at the proximal electrode differed significantly. The short-term administration of TJ-43 relieved symptoms and reduced the distal esophageal acid exposure through improved esophageal acid clearance.

Laparoscopically assisted anorectoplasty using perineal ultrasonographic guide: a preliminary report.

BACKGROUND/PURPOSE: As minimal invasive surgery, laparoscopically assisted anorectal pull-through has been reported with new devices. However, it is not easy to create an accurate pull-through canal (PTC) because of the narrow space between the urethra and puborectal sling. The authors describe a new method using perineal ultrasonography. METHODS: The rectourethral prostatic fistula was dissected laparoscopically. Externally, electrostimulation identified the center of the muscle contraction, over which a 1.2-cm skin incision was made, and the lower part of PTC was created by hemostat forceps guided by electrostimulation. An ultrasonographic probe applied to the perineum demonstrated the urethra, and the forceps was advanced behind the urethra into the pelvic cavity using the ultrasonographic guide. Anorectal pull-through was performed after dilatation of the PTC with dilators. RESULTS: The authors applied this procedure in 5 cases of male high and intermediate anomalies. Surgical damages to the urethra and the levator and vertical muscles were not encountered. Postoperative fluoroscopic study demonstrated good anterior angulation and intact contraction and relaxation of those muscles. CONCLUSION: The combination of laparoscopic dissection, pinpointing the center of anal sphincter by electrostimulation, and identification of the urethra by ultrasonographic images from the perineum facilitated creation of appropriate PTC in the muscle complex.

Cow's milk allergy presenting Hirschsprung's disease-mimicking symptoms.

The authors present a neonatal case of allergic colitis, which manifested the difficulty of spontaneous defecation and irregular narrowing of distal rectum in contrast enema. Rectal suction biopsy showed positive acetylcholinesterase activity. These clinical, radiological and histological findings were indistinguishable from Hirschsprung's disease. Gastrointestinal symptoms were improved by the cessation of cow's milk formula. The present findings may impact on the less recognizable gastrointestinal manifestation of allergic colitis.

Follow-up studies of anorectal malformations after posterior sagittal anorectoplasty.

BACKGROUND/PURPOSE: There are few follow-up studies comparing posterior sagittal anorectoplasty (PSARP) with conventional procedures for patients with anorectal malformations (ARM). The authors have examined retrospectively postoperative anorectal function of patients with ARM treated with PSARP compared with those treated with conventional methods. METHODS: Anorectal function in 23 patients with high and intermediate type anorectal malformations (PSARP group), who underwent PSARP more than 4 years previously, were assessed by Kelly's clinical scoring system and objective studies. These results were compared with those in 14 cases (5 high and 9 intermediate type cases; control group), who underwent other conventional surgical procedures. RESULTS: Using Kelly's clinical scoring system, scores of the PSARP group compared with the control group were good in 48% versus 21%, fair in 48% versus 58%, and poor in 4% versus 21%, respectively. Barium enema studies suggested better anorectal sphincteric function in patients with high anorectal malformation in the PSARP group. Magnetic resonance imaging (MRI) studies showed more correct placement of the rectum through the striated muscle complex in the PSARP group at the I-line level. Manometric studies showed no difference in maximum resting pressure, anal canal length, and the incidence of anorectal reflex between the two groups. CONCLUSIONS: The favorable results of MRI and barium enema studies can be explained by direct visualization of the striated muscle complex with the aid of electrical stimulation as well as no harmful effects of amputation of the sphincter muscle in PSARP. However, manometric studies suggest anorectal function in patients with high and intermediate anorectal malformations is limited even after PSARP. Long-term postoperative follow-up with adequate bowel management is required for all patients with high or intermediate anorectal malformation.