Search

Background: Intestinal volvulus in a neonate, complete or segmental, is a true surgical emergency, which when the diagnosis is delayed can bring detrimental results. The aim of this study was to describe the clinical characteristics of intestinal volvulus during the neonatal period by comparing total midgut volvulus (TMV) and segmental volvulus (SV)., Methods: The medical records of 44 neonates who were operated on for intestinal volvulus from 1993 to 2019 were retrospectively reviewed. The patients were divided into TMV and SV groups, and clinical features were compared., Results: Operations were performed on 27 patients for TMV and 17 for SV. All cases of TMV were associated with intestinal malrotation, while those with SV were not. Gestational age, birth weight, and ratio of prematurity showed no differences between the groups. Preoperative diagnosis of TMV or SV was possible in 23 and 5 (85% and 29%) cases, respectively. Intestinal resection was required in 16/17 patients (94%) with SV, while it was required in 5/27 (19%) patients with TMV. When bowel resection was performed in TMV, all but one patient suffered from short bowel syndrome leading to two mortalities, while SV group showed good recovery., Conclusion: Diagnosis of SV before laparotomy can be difficult. Even though performing intestinal resection in SV neonates is highly likely, it shows a favorable outcome. TMV neonates are less likely to undergo intestinal resection; however, when bowel ischemia is present, significant morbidity can occur., Type of Study: Treatment study., Level of Evidence: Level IV., (Copyright © 2020 Elsevier Inc. All rights reserved.)

Objective: Neonatal gastric perforation is a rare and serious issue. This study aimed to highlight the vital clinical features and identify prognostic factors in such cases., Design, Setting, Patients, Interventions, and Measurements: Medical charts from January 1997 through December 2008 were reviewed retrospectively. Neonates with a diagnosis of gastric perforation were included., Results: Thirteen patients were identified with a male:female ratio of 9:4. Five (38%) were preterm infants. The mortality rate was 30% (4/13), and the median age of onset was 3 days (range: 1-14 days). The most common presenting sign was abdominal distension, followed by respiratory distress and vomiting. Except for one patient in whom gastric perforation was diagnosed during surgical repair for gastroschisis, all patients had pneumoperitoneum on admission; 70% and 46% of patients had peritonitis and sepsis, respectively. Concomitant gastrointestinal (GI) tract anomalies or disorders included ischemic bowel/necrotizing enterocolitis (5 patients), intestinal malrotation (2), duodenal web (1), hiatal hernia (1), and gastroschisis (1), which necessitated secondary operations during hospitalization in 5 patients. Seven patients had leukopenia on admission, and 9 developed thrombocytopenia in the following 48 h. All patients who died presented with leukopenia on admission and thrombocytopenia in the following 48 h, yielding sensitivity and specificity rates of 100% and 67%, respectively., Conclusions: Neonatal gastric perforation is often concomitant with GI anomalies or inflammatory/infectious disease. Patients who were outborn and those with leucopenia, peritonitis, and thrombocytopenia development within 48 h were at risk for poor outcome., (Copyright © 2015 Elsevier Inc. All rights reserved.)

Introduction: Currarino syndrome is a type of caudal regression syndrome characterized by the association of hemisacrum, anorectal malformation and presacral mass. Only few studies on small series report the incidence of urinary dysfunction in Currarino syndrome. Our aim was to evaluate the urological outcome in patients with Currarino syndrome., Patients and Methods: We retrospectively reviewed all Currarino syndrome patients treated in our institution. Of 20 patients, we could evaluate the urological outcome in 16. This group of patients underwent clinical, radiological and urodynamic evaluation., Results: All 16 patients had a sacral defect, fourteen of them presenting a presacral mass (87.5%), eight a tethered cord (50%), and 7 anorectal malformations (43.7%). Eight patients underwent neurosurgical treatment for neural tube defects. In 14 patients, the presacral mass was resected. One case presented detrusor overactivity, 2 recurrent urinary tract infections and 2 vesicoureteral refluxes. Both patients with lipomyeloschisis had a neuropathic bladder. All the other patients could void the bladder spontaneously. Renal function was normal in all., Conclusion: Currarino syndrome is a rare congenital disorder presenting a variable phenotype. Urological outcome is good in the majority of patients., (Copyright © 2014 Elsevier Inc. All rights reserved.)

In patients with intestinal malrotation, Ladd bands are known to cause obstruction of the duodenum and small bowel. However, acute obstruction of the colon from Ladd bands has not been previously described. We report a case of complete obstruction of the hepatic flexure of the colon secondary to a Ladd band in a previously healthy teenage boy.

This report describes a case of congenital thickening and shortening of the round ligament of the liver, a rare cause of gastric outlet obstruction. There are few published cases in the literature and these were generally diagnosed at a late stage and were mostly among adults. The patient underwent exploratory laparotomy, which showed gastric compression in the distal antrum, close to the pylorus, caused by the round ligament, which was shortened and thickened. The round ligament was suture ligated and divided, and the patient's symptoms resolved. Comments on this abnormality and its rarity are presented.

A unique case is reported of bronchopulmonary foregut malformation with associated benign teratoma. Antenatal Doppler ultrasound scan findings and postnatal management are described. We discuss the appropriate radiologic investigations and the necessity for surgical intervention. The possible embryogenesis is hypothesized.

Background/purpose: Megarectum in association with anorectal malformation contributes to chronic constipation and fecal incontinence. Resection of megarectum in anorectal malformation improves bowel function, but neuropathy and poor sphincter quality may affect the outcome of fecal continence adversely. The aim of this study was to evaluate the benefits of resection of megarectum in anorectal malformation and to ascertain the impact of anal sphincter quality and neuropathy on the outcome., Methods: We studied 62 children with intractable fecal incontinence after repair of anorectal malformation between January 1991 and January 2005. All patients were investigated with anorectal manometry and anal endosonography under ketamine anesthesia. On endosonography, an intact or scarred internal anal sphincter (IAS) was classified as good and a fragmented or absent IAS as poor. On manometry, a resting anal sphincter pressure equal to or more than 30 mm Hg was classified as good and a lower pressure as poor. Functional assessment of fecal continence was done before and after excision of megarectum using a modified Wingfield scores., Results: Sixteen children had excision of megarectum with median age of 9 years (range, 2-15 years) and postoperative follow-up of 5 years (range, 1-10 years). Seven had formation of antegrade continent enema stoma before excision of megarectum. Children were classified into three groups of anomalies: low (n = 6), intermediate (n = 4), and high (n = 6). All children were incontinent of feces. After excision of megarectum, of the 9 children with good IAS and no neuropathy, 7 became continent of feces. Of the remaining 7 children, 4 had poor IAS and 3 had neuropathy, 5 of whom required an antegrade continent enema stoma to be clean., Conclusion: Excision of megarectum in children who had previous repair of anorectal malformation results in fecal continence in the presence of a good IAS and absence of neuropathy. Patients with a poor IAS or neuropathy will often require artificial means of fecal continence.

Congenital absence of the portal vein (CAPV) requires liver transplantation when encephalopathy develops. However, transplantation has technical difficulties because no collateral circulation exists except for the portosystemic shunt. Ligating the shunt will cause disastrous mesenteric venous congestion. We report a 19-month-old female infant with CAPV, who had portosystemic encephalopathy and cardiac failure, and underwent living donor liver transplantation with a partial clamp technique using a vein graft. This is the first case of successful liver transplantation for CAPV with cardiac failure.

Purpose: A new generation of magnetic resonance imaging scanner offers a 3-dimensional magnetic resonance cholangiopancreatography (MRCP) with very high spatial resolution using a non-breath-hold technique with prospective navigator gating. The study aimed to investigate the new technology of the MRCP in infants with suspected pancreaticobiliary diseases., Methods: Seven patients (4 girls, 3 boys; mean age, 2.8 years; range, 3 months to 5.6 years) were examined. All patients underwent magnetic resonance imaging examination using a 1.5-T whole-body scanner (Magnetom Avanto, Siemens Medical, Erlangen, Germany). For MRCP, a heavily T2-weighted (time to echo, 678 ms) 3-dimensional turbo spin echo in coronal plane was performed with the voxel size of 1 x 1 x 1.5 mm. To enable non-breath-holding imaging, a diaphragm navigator sequence was simultaneously carried out. The MRCP findings were compared prospectively with the endoscopic retrograde cholangiopancreatography and/or with the surgical findings in 6 of 7 cases. In 1 patient, the follow-up was considered reference standard., Results: The high spatial resolution of the images without breathing artifacts allowed an excellent demonstration of all relevant pancreaticobiliary ducts and of the pathologic findings. All patients (n = 4) with choledocholithiasis were identified through MRCP. The finding of a high pancreaticobiliary junction (n = 3) was confirmed by endoscopic retrograde cholangiopancreatography in all cases. The length of the common channel ranged from 16 to 24 mm. In 1 patient, the long common channel was associated with a choledochal cyst. In a 1.7-year-old boy with recurrent abdominal pain and mild pancreatitis, the rare case of a gastric duplication with connection to the main pancreatic duct was diagnosed. A regular gastric wall with normal mucosa and parts of the duodenal wall with a circular layer of muscle (pylorus-like) were found in histology after surgical resection of the mass., Conclusion: The newly developed technique may be a reliable tool in the workup of pancreaticobiliary diseases in infants.

Agenesis of the dorsal mesentery in association with jejunal atresia is a well-recognized congenital anomaly. Only few reports exist in the English literature of children with agenesis of the dorsal mesentery without small bowel atresia. Herein, we report a child with total mesenteric agenesis presenting with midgut volvulus owing to internal herniation of the small bowel through a mesenteric defect, with normal fixation and rotation of the bowel.

Bronchopulmonary foregut malformations are uncommon anomalies, and their association with diaphragmatic eventration is rare. This report presents 2 cases with this association. Embryologically, a simultaneous disturbance in the development of pulmonary vessels and developing esophagotracheal septum may explain the etiopathogenesis of this association. Abnormal budding along the developing foregut tube may lead to foregut cysts. In both cases, the bronchopulmonary foregut malformations were excised, and the diaphragm was repaired. The results of surgery were gratifying in both cases.

Imperforate anus and malrotation rarely occur together. The conjoint occurrence of these with Hirschsprung's disease is exceedingly rare, but failure to recognize its existence may lead to catastrophic results. We present a case in which awareness to this possibility caused early detection and avoidance of possible complications.

Background/purpose: Colonic atresia (CA) is one of the rarest causes of neonatal intestinal obstructions, and no large series can be reported. Therefore, we did perform a retrospective clinical trial to delineate our CA cases and carry out a literature survey., Methods: We reviewed the charts of CA cases treated in our center between 1992 and 2002. We aimed to collect all reported cases in Medline, and personal communications with the authors of published series were used to reach the missing data., Results: The chart review revealed 9 newborns with CA treated in our center (6 cases of type III, 2 cases of type II, and 1 case of type IV). These accounted for 3.7% of all gastrointestinal atresias managed in our center. Of the CA cases, 3 were isolated and 6 had at least one or more associated congenital anomalies. The preferred surgical technique at the initial treatment of CA was performing a proximal stoma and distal mucous fistula in an average of postnatal 59.4 hours. The literature survey enabled us to reach 224 cases of CA, including our cases., Conclusions: Because of the low incidence of CA, delay in diagnosis and treatment may occur. The mortality is statistically higher when the surgical management is performed after 72 hours of age. However, the prognosis of CA is satisfactory if diagnosis and surgical management could be made promptly and properly.

Background: Fecal accumulation, constipation, soiling, and incontinence are common sequelae after repair of anorectal malformations (ARMs) in children. It is believed that besides the abnormalities of sacral roots, certain inherent abnormalities of the myenteric plexuses may play an important role in the final outcome after definitive repair., Methods: This study was conducted to investigate the distribution of neuron-specific enolase (NSE), vasoactive intestinal peptide (VIP), and substance P (SP)-100 neurotransmitters in the rectosigmoid and fistulous tract of the ethylenethiourea-treated rat with ARMs., Results: ARMs were induced by administering 1% ethylenethiourea (125 mg/kg) on gestational day 10, and the litter was harvested on gestational day 21 by cesarean section. Forty-eight controls and 63 with ARMs (46 high-type and 17 low-type) were recovered. Whole-mount preparations of each rectosigmoid and fistulous communication between the rectum and genitourinary tract were stained with fluorescent antibodies against NSE, VIP, and SP-100. The tissues were counterstained with Eriochrome black-T and methyl green dyes to improve the visualization of the myenteric plexuses., Conclusions: The immunoreactivity of NSE, VIP, and SP-100 was markedly reduced in the rectum and fistulous tract of high-type ARMs and slightly reduced in low-type ARMs compared with controls. Intramural nerves stained by VIP and SP-100 antisera were decreased in both types of ARM, indicating that both inhibitory and excitatory motor neural elements were affected, and this may explain the distal colonic dysmotility seen postoperatively in both high and low ARMs.

Purpose: Because malrotation most commonly presents in infants, treatment recommendations for older children (>1 year) have been based on data obtained from small case series. The purpose of this study was to use a large national database to determine the clinical significance of older children presenting with malrotation to develop treatment recommendations for this group., Methods: Records of children undergoing a Ladd's procedure were identified in the Kids' Inpatient Database, an administrative database that contains all pediatric discharges from 27 states during 2000. Patient characteristics, associated diagnoses, operations performed, and mortality were evaluated. Discharge weighting was used to obtain a national estimate of the number of children older than 1 year treated for malrotation., Results: Two hundred nineteen older children (>1 and <18 years) undergoing a Ladd's procedure were identified in the database. One hundred sixty-four (75%) of these patients were admitted for treatment of malrotation, whereas most of the remaining 55 patients (25%) were admitted for another diagnosis and underwent a Ladd's procedure incidental to another abdominal operation. Seventy-five patients underwent a Ladd's procedure during an emergency admission. Thirty-one patients had volvulus or intestinal ischemia, 7 underwent intestinal resection, and 1 patient died. Based on case weightings, it was estimated that 362 older children underwent a Ladd's procedure for symptoms related to malrotation in 2000 in the United States (5.3 cases per million population)., Conclusions: These findings provide support for performing a Ladd's procedure in older children with incidentally found malrotation to prevent the rare but potentially devastating complications of this anomaly.

Purpose: Rectal prolapse is a known postoperative problem in children with anorectal malformations. The aims of this study were to determine the incidence of significant rectal prolapse (>5 mm), to objectively quantify its predisposing factors, and to offer recommendations as to its prevention and surgical treatment., Methods: The authors reviewed their series of 1619 patients with anorectal malformations; 1169 underwent primary posterior sagittal anorectoplasty (PSARP) at their institution between 1980 and 2002, and complete records were available for 833. The series was analyzed for incidence of prolapse, type of anorectal malformation, status of the sacrum, muscle quality, associated vertebral and spinal anomalies, and postoperative constipation. A specific technique for prolapse repair was used., Results: Of 833 patients, 45 developed significant rectal prolapse (3.8%). The mean age at the time of PSARP was 0.73 years (range, 0.19-5 years). The average time to recognition of prolapse following PSARP was 13.1 months. Of these 45 patients, 32 required surgical repair and of those, 3 required a second surgical repair. The incidence of prolapse varied by complexity of anorectal defect: cloaca (6.2%), rectobladder neck fistula (6.8%), rectourethral fistula (5.4%), rectovestibular fistula (1.2%), rectal atresia (0%), and rectoperineal fistula (0%). There was a significantly increased incidence of prolapse in patients with a low muscle quality score and in patients with vertebral anomalies (20% vs 3.2%). The presence of a tethered cord and an abnormal sacral ratio did not correlate with an increased incidence of prolapse. Twenty-two patients developed prolapse following colostomy closure, and of these, 12 (55%) suffered from constipation., Conclusions: The overall incidence of significant rectal prolapse following PSARP is low. Prevention of prolapse with the PSARP technique may be because of key technical steps. Patients with higher anorectal malformations, poorer muscle quality, and vertebral anomalies had a greater risk of developing postoperative rectal prolapse. The presence of tethered cord and quality of the sacrum were not predictive of postoperative prolapse. Constipation seems to be a factor in the development of prolapse.

Background: The aim of this study was to review the outcome of surgical management of various types of perineal masses encountered in patients with anorectal malformations (ARM)., Methods: Retrospective review from 2 large pediatric anorectal referral centers., Results: Twenty-two patients with a perineal mass were identified in more than 2000 patients treated for an ARM over a 15-year period. The 22 patients (4 men) represented all levels of severity of ARMs. The lesions were of 3 types: lipomas (n = 10), vascular anomalies (n = 4), and hamartomas/choristomas (n = 8). The lipomas were carefully removed from between the muscle fibers during the posterior sagittal anorectoplasty. The vascular anomalies (3 of 4 were hemangiomas) underwent magnetic resonance imaging preoperatively, but none were found to invade deeply and all were excised at the time of the posterior sagittal anorectoplasty. The hamartomas/choristomas all occurred in women, and 50% arose as a pedunculated mass from the vulva. The lesions contained tissues such as glia, osteoid, nephrogenic rests, and endocervical-type mucosa. One was initially misinterpreted as a teratoma, prompting a wider excision. This and all subsequent patients have been correctly diagnosed pathologically as having either hamartomas or choristomas, which were not widely excised. Follow-up ranges from 5 months to 12 years. Six of the 10 lipoma patients are continent. One vascular anomaly was re-excised and there was minor wound separation in another. None of the hamartoma/choristoma lesions recurred., Conclusion: The presence of unusual perineal masses can add to the complexity of ARMs; however, most of these lesions can be carefully excised with preservation of the muscle complex and ultimate continence. Hamartomatous lesions can be mistaken for teratomas but do not require aggressive excision with clear margins.

Background/purpose: The aim of this study was to review the management of the gastrointestinal tract in cloacal exstrophy and to assess the effect of spinal dysraphism on nutritional outcome., Methods: Twenty-two patients with cloacal exstrophy were reviewed retrospectively. Gastrointestinal and spinal anomalies were documented. The need for nutritional supplementation was recorded. Weights at 1 and 5 years of age were used to assess growth in childhood., Results: Ten patients were treated initially with ileostomy, 7 with colostomy, and 3 without stoma. Two patients died before surgical reconstruction. Total parenteral nutrition (TPN) was utilized in 8 infants for a median of 10 days (range, 5 to 200). Three patients had complications with colostomies requiring conversion to ileostomy. Patients with an ileostomy required more nutritional supplementation compared with patients with a colostomy (50% v 28%). High-output stoma losses were more common in patients with an ileostomy (40% v 14%). Fourteen patients (67%) with spinal dysraphism had a higher incidence of failure to thrive in the first year of life (69% v 26%) and multiple episodes of enteritis (40% v 25%). Two neonates with duodenal atresia and small bowel deletion died within the first month of life. One patient with short bowel syndrome died of TPN-associated liver disease at 6 months of age. There were no other deaths., Conclusions: The gastrointestinal tract contributes significantly to the morbidity and mortality in cloacal exstrophy. Nutritional supplementation is more frequently required in patients with an ileostomy. Stoma complications were higher in those with a colostomy. Morbidity is high in patients with spinal dysraphism.

Background: Evidence supporting routine surgery for asymptomatic tethered cord in patients with anorectal malformation (ARM) is, at best, speculative. The authors therefore examined whether untethering is indicated for asymptomatic tethered cord in patients with ARM., Methods: A retrospective analysis of all patients with ARM (n = 223) between 1992 and 2002 was conducted. During the same period, 435 patients had surgery for tethered cord., Results: Tethered cord was detected radiologically in 22 (9.8%); 8 patients with a low conus, and 14 with a low conus with and thickened filum. Seven of 22 patients underwent untethering; 3 prophylatic (14%) and 4 for neuro/motor function deficits (18%). All 4 symptomatic patients had significant clinical improvement in their neuro/motor functions after surgery. However, bowel and urinary functions remained unchanged in all 7 patients with a mean follow-up of 6.4 years (range, 4 to 8 years). Fifteen patients with radiologically diagnosed tethered cord remain asymptomatic with a mean follow-up of 2.7 years (range, 8 months to 10 years)., Conclusions: Neuro/motor functions clearly improved with surgery in symptomatic patients. However, bowel and urinary functions remained unchanged after surgery. Only 4 ARM patients with tethered cord required surgery, whereas prophylactic surgery appears to have minimal benefit. Expectant conservative approach in the management of asymptomatic tethered cord patient appears to be safe.

The authors describe a technique for construction of a continent catheterizable stoma from distal defunctionalized colon in a patient with imperforate anus, urethral atresia, and sacral agenesis.

Purpose: The aim of this study was to determine the presence of specific clinical symptoms in intestinal neuronal dysplasia (IND) and whether it correlates to the severity of histopathologic findings., Methods: A group of 44 severe IND and a group of 16 mild IND patients diagnosed by means of a histochemical rectal biopsy were compared with a group of 37 patients with functional constipation (FC) with normal rectal biopsy results., Results: Patients with severe IND began their symptoms at an earlier age than those with mild IND and FC (5.2 +/- 112 months v 17.5 +/- 23 months and 22.5 +/- 21.8 months, respectively; P <.001). The presence of intestinal obstruction symptoms was more frequent in severe IND patients than in mild IND and FC patients (45.5% v 18.8% v 2.7%, respectively; P <.001). The presence of a fecaloma and soiling were less frequent in the severe IND group than in mild IND and FC groups (20.5% v 56.3% v 59.5%, respectively; P <.001 and 15.9% v 31.3% v 59.5%, respectively; P <.001). Barium enema results showed a lower incidence of rectosigmoid distension in severe IND if compared with mild IND and FC groups (45.5% v 57.1% v 96.9%; P <.001). Internal sphincter relaxation was absent frequently in the severe IND group compared with the FC group (47% v 26.9%, respectively; P <.05)., Conclusions: Intestinal neuronal dysplasia is a distinct histopathologic and clinical entity. Its clinical, radiologic, and manometric presentation correlates to the severity of histochemical findings., (Copyright 2002, Elsevier Science (USA). All rights reserved.)

Purpose: The authors devised computerized equipment for use in the biofeedback therapy in the management of fecal continence after surgery for anorectal malformations., Methods: The therapy was used for two to eight sessions in 14 children (11 who had high-type anomalies and three who had intermediate-type anomalies). The ages ranged 5 to 14 years. A control group of 17 children, aged 5 to 11 years, who had encopresis, was also treated with the same biofeedback therapy., Results: Clinical improvement was noted in 5 of the 14 (36%) children who had fecal incontinence, and in 15 of the 17 children (88%) who had encopresis. Both in patients who had fecal incontinence and in those who had encopresis, anal resting pressures were not affected by biofeedback therapy. Furthermore, the anal resting pressure in children who had fecal incontinence was significantly lower than that in children who had encopresis. However, anorectal manometry showed that the biofeedback therapy improved voluntary sphincter function and rectal sensation in both groups., Conclusion: Biofeedback therapy appears to be effective in most children who have encopresis whose sphincter function is intact, and in some children who have fecal incontinence after surgery for anorectal malformations.