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PubMed2006

Colonic manometry as predictor of cecostomy success in children with defecation disorders.

PURPOSE: The aim of this study was to define the predictive value of colonic manometry and contrast enema before cecostomy placement in children with defecation disorders. METHODS: Medical records, contrast enema, and colonic manometry studies were reviewed for 32 children with defecation disorders who underwent cecostomy placement between 1999 and 2004. Diagnoses included idiopathic constipation (n = 13), Hirschsprung's disease (n = 2), cerebral palsy (n = 1), imperforate anus (n = 6), spinal abnormality (n = 6), and anal with spinal abnormality (n = 4). Contrast enemas were evaluated for the presence of anatomic abnormalities and the degree of colonic dilatation. Colonic manometry was considered normal when high-amplitude propagating contractions (HAPC) occurred from proximal to distal colon. Clinical success was defined as normal defecation frequency with no or occasional fecal incontinence. RESULTS: Colonic manometry was done on 32 and contrast enema on 24 patients before cecostomy. At follow-up, 25 patients (78%) fulfilled the success criteria. Absence of HAPC throughout the colon was related to unsuccessful outcome (P = .03). Colonic response with normal HAPC after bisacodyl administration was predictive of success (P = .03). Presence of colonic dilatation was not associated with colonic dysmotility. CONCLUSION: Colonic manometry is helpful in predicting the outcome after cecostomy. Patients with generalized colonic dysmotility are less likely to benefit from use of antegrade enemas via cecostomy. Normal colonic response to bisacodyl predicts favorable outcome.

Adolescent↗

PubMed2006

Minimal access surgery in neonates and infants.

BACKGROUND: Minimal access surgery (MAS) in small infants carries an important consideration. The tolerance of these small babies and the assumed physiological effect of MAS, in addition to the required anesthetic and surgical skills, have made it difficult to perform these types of procedures in many international centers. The present article reviews our experience with MAS in neonates and infants in the first year of life. METHODS: The medical records of all neonates and infants (<1 year) who underwent MAS over a period of 3 years were retrospectively reviewed for demographic information, procedures, operative time, complications, outcomes, and follow-up. Most of the operations were performed with 3-mm instruments and scopes and mean insufflation pressure of 10 mm Hg (range, 4-15 mm Hg). RESULT: Seventy neonates and infants were included in this study: 19 females and 51 males. The weight ranged from 1.3 to 8.2 kg (mean, 4.3 kg). The mean age was 93 days (range, 1 day to 12 months). Twenty-four (34%) were neonates (first 30 days of life). Procedures performed included repair of tracheoesophageal fistula, lobectomy, repair of diaphragmatic or hiatus hernias, pull-through for imperforated anus and Hirschsprung's disease, plication of the diaphragm, Kasai procedure, excision of choledochal cyst, pyloromyotomy, Ladd's procedure, and reduction of intussusceptions. There were 2 conversions, both in neonates with tracheoesophageal fistula. All patients tolerated the procedure very well, with lesser degrees in neonates undergoing thoracoscopic procedures. Two neonates had postoperative hypothermia (<35 degrees C) and 1 neonate had high PCO2 postoperatively. There was 1 mortality and no morbidities. The follow-up ranged from 1 month to 3 years (mean, 19 months). CONCLUSION: Minimal access surgery in neonates and infants is safe and well tolerated. Intraoperative monitoring of end-tidal CO2 and core temperature is essential in avoiding unwanted effects of performing these procedures, especially in neonates.

Feasibility Studies↗

PubMed2006

Colonic triplication associated with anorectal malformation: case presentation of a rare embryological disorder.

Tubular colonic triplication is an extremely rare hindgut malformation, with only 2 reports in the literature to date. The present authors describe the new and unusual case of a boy born with an imperforate anus, rectovesical fistula, and 3 distinct left colons. The bladder was divided by an incomplete septum. Prenatal ultrasound suggested colonic duplication. Surgical management involved resection of the triplicated segment and posterosagittal anorectal pull through.

Adult↗

PubMed1998

Accurate detection of anomalies by routine ultrasonography in an indigent clinic population.

OBJECTIVE: The study's objective was to determine the accuracy of routine ultrasonography in the detection of anomalies in an indigent clinic population by sonographers trained to perform complete anatomic screens. STUDY DESIGN: This is a retrospective study of women undergoing obstetric ultrasonography between 15 and 26 weeks' gestation during an 18-month period. Ultrasonography was performed with an ATL Ultramark 4 (ATL Ultrasound, Bothell, Wash) in the resident clinic by a rotating group of sonographers who each spent 80% of the time performing advanced sonography and 20% screening. Images were reviewed by attending physicians at the end of each day. Abnormalities were followed up with ultrasonography by an attending physician. Obstetric and neonatal charts were reviewed in all cases. RESULTS: There were 901 women scanned (44 others were unavailable for follow-up, and complete data were unavailable in 22 cases). Twenty-one percent of all patients were referred for advanced scans; in half the only indication for referral was an abnormal ultrasonographic result. There were 28 anomalies (3.1%). Anomalies that were undiagnosed were a 5-mm ventricular septal defect, transposition of the great arteries with ventricular septal defect, extralobar sequestration, misshapen ear, clubfoot, imperforate anus, 1 x 1-cm skin-covered meningomyelocele, and an uncharacterized dysmorphic syndrome. The sensitivity, specificity, and negative and positive predictive values of the screening ultrasonography were 71.4%, 99.4%, 99.1%, and 80%, respectively (P < .0001). Of patients in whose fetuses anomalies were detected, 30% elected termination of pregnancy and 1 patient whose fetus had trisomy 18 elected no fetal monitoring in labor, resulting in a stillbirth. Two neonates required surgery and 2 died in the immediate neonatal period. There were 5 suspected anomalies at screening that were excluded by targeted scan. CONCLUSION: Routine screening ultrasonography performed by well-trained sonographers is extremely accurate in the detection of anomalies in an indigent population. It allows women counseling and options that they otherwise might not be offered.

Abortion, Induced↗

PubMed2002

p63 Coordinates anogenital modeling and epithelial cell differentiation in the developing female urogenital tract.

p63 is a p53 homologue required for cutaneous development that is expressed in immature squamous epithelium and reserve cells of the cervix. Humans with p63 mutations exhibit defects in limb, accessory organ (skin appendage, breast, prostate), and genitourinary development. Because p63 expression patterns imply a strong role of the gene in the female genital tract development, newborn female p63-/-, +/-, and +/+ mice were examined in situ, dissected, and compared. Nuclear p63 protein was localized to the skin, vagina, bladder, urethra, and basal columnar cells of the caudal uterus in p63+/+ and +/- animals. p63-/- mice exhibited abnormal genital morphogenesis with hypoplastic genitalia, a single cloacal opening, and persistence of columnar epithelium at lower genital tract sites that normally undergo squamous and urothelial differentiation. The defects observed support p63-dependent pathways of genital tract development that permit externally, ectodermal basal cell replenishment integral to reciprocal epithelial stromal signaling, urorectal septation, and modeling of the external genitalia; and internally, the emergence of basal epithelial cell populations capable of divergent epithelial cell differentiation in the vagina, cervix, and urinary tract. Defects in the first pathway explain imperforate anus, vaginal septum, genital hypoplasia, and micropenis reported in humans with p63 mutations. The second is necessary for the generation of multipotential reserve cells in the cervix and may be operative in other epithelial stromal interactions integral to the emergence of uterine basal cells later in life.

Anal Canal↗

PubMed1991

Ejaculatory duct obstruction in subfertile males: analysis of 87 patients.

OBJECTIVE: To study the causes, presentation, and treatment of ejaculatory duct obstruction in subfertile males. DESIGN: Collaborative retrospective study of clinical experience collected by two urologists over a 15-year period. SETTING: National Health Service and Private Care Hospitals. PATIENTS, PARTICIPANTS: Subfertile males with azoospermia (n = 67), very severe oligozoospermia (n = 17), oligozoospermia (n = 1), or normal sperm concentration (n = 2) in small volume ejaculates with acid pH and little or no fructose. INTERVENTIONS: Exploration of scrotum with vasogram and testicular biopsy, plus reconstruction if possible. MAIN OUTCOME MEASURES: Follow-up seminal analysis and occurrence of pregnancy in female partners. RESULTS: The causes were: müllerian duct cyst (n = 17); wolffian duct malformation (n = 19); previous surgical trauma (e.g., imperforate anus) (n = 15); previous genital infection (n = 19); tuberculosis (n = 8); megavesicles (pathological dilatation of vesicles and ampullae of unknown cause) (n = 8); and carcinoma of prostate (n = 1). After incision of Mullerian duct cysts, five pregnancies were produced. Five pregnancies occurred in the other groups using a variety of surgical techniques. CONCLUSIONS: Routine vasography has shown that ejaculatory duct obstruction is not as rare as previously thought. The diagnosis should not be missed because the condition is simple to correct surgically in certain cases.

Constriction, Pathologic↗

PubMed2003

Primary reversed gastric tube reconstruction in long gap esophageal atresia.

BACKGROUND/PURPOSE: Esophageal reconstruction in long-gap esophageal atresia (EA) poses a technical challenge with several surgical options. The purpose of this study was to review the authors' experience with the reversed gastric tube (RGT) in esophageal reconstruction. METHODS: This series describes 7 babies with pure EA treated at 2 centers between 1989 and 2001. Data, gathered by retrospective chart review, included clinical details of the esophageal and associated malformations, technique and timing of repair, early and late complications, and long-term follow-up. Institutional review board (IRB) approval of this study has been obtained. RESULTS: Seven babies were included. Associated malformations were present in 4: trisomy 21 in 2 and imperforate anus in 2. After gastrostomy tube placement, patients were treated with gastrostomy tube feedings and continuous upper pouch suction. Median gap length was 5.5 vertebral segments (range, 3 to 9). RGT with a posterior mediastinal esophagogastric anastomosis was performed at median age of 62 days (range, 38 to 131). There were no anastomotic leaks. Three patients had strictures, one required resection. Exclusive oral nourishment was achieved in 5 patients by 6 months of age. At last follow-up (mean, 4.5 years), 6 patients were receiving oral nutrition exclusively, and all were maintaining growth curves. CONCLUSIONS: In long gap EA, early esophageal reconstruction using an RGT can be performed with minimal morbidity and promising short-term results.

Anastomosis, Surgical↗

PubMed1993

Cloacal exstrophy: experience with 20 cases.

Exstrophy of the cloaca, a rare anomaly, is among the most complex malformations of infancy. Features include omphalocele, imperforate anus, and exstrophy of two hemibladders, between which lies the everted cecum. A small colon ends blindly in the pelvis, and the terminal ileum often prolapses out of the exposed cecum. Formerly, most of these infants died. From 1974 to 1992, 20 of these patients were treated, the majority in the past decade. Six were managed primarily; 14 were referred after prior surgery elsewhere. Current treatment of a newborn with cloacal exstrophy includes closure of the omphalocele, separation of the gastro-intestinal tract from the hemibladders, and closure of the two hemibladders as a single viscus. The colon can be pulled through in some cases, either immediately or later (6 cases). The bladder must be augmented in all cases to provide adequate volume and compliance; stomach is ideal for this (10 cases). A bladder continence mechanism must be constructed by narrowing the outlet (9 cases), or inserting a reversed small bowel nipple (7 cases). Intermittent catheterization is needed for the patient to empty the reconstructed bladder. Although two thirds of these patients are genetic males, they should be raised as females because they lack adequate tissue to construct a phallus. Magnetic resonance imaging of the spine has shown tethering of the cord in all patients; most were released neurosurgically. Lessons provided by these cases demonstrate that a satisfactory surgical and social outcome can be achieved today in most children with cloacal exstrophy.

Abnormalities, Multiple↗

PubMed1993

Congenital duodenal obstruction: a 32-year review.

Although survival in infants with congenital intestinal obstruction has improved, duodenal obstruction continues to present unique challenges. One hundred thirty-eight newborns and infants (aged 0 to 30 days) were treated for congenital duodenal obstruction. Sixty-five were boys and 73 were girls. Sixty-one (45%) were premature. Forty-six had an intrinsic defect (atresia, web, stenosis, or duplication), 64 had an extrinsic defect (annular pancreas or malrotation with congenital bands), while 28 had various combinations of these. Presenting signs included vomiting (90%, bilious in 66%), abdominal distention (25%), dehydration (24%), and weight loss (17%). Although plain film abdominal x-ray was diagnostic in 58%, upper and/or lower gastrointestinal contrast studies were obtained in 71% of infants to confirm diagnosis. Thirty-eight percent of patients had associated anomalies, including Down's syndrome (11%), cardiac defects, other atresia, other trisomy syndrome, imperforate anus, and central nervous system anomalies. Fourteen patients (10%) had 3 or more other anomalies, many of which required additional surgical therapy. The operative repair of the various defects included Ladd's procedure for malrotation (31%), duodenoduodenostomy (14%), duodenojejunostomy (22%), gastrojejunostomy or gastroduodenostomy (4%), excision of the web and duodenoplasty (3%), or combination of the above (22%). Gastrostomy was placed in 61%. One hundred twenty-eight patients survived (93%). The causes of death were combinations of sepsis, pneumonia, brain hemorrhage, short bowel, and cardiac anomaly. Eight of 10 (80%) who died had other serious anomalies. Twenty patients (14%) required reoperation 5 days to 4 years postoperatively for obstructing lesions (5), wound dehiscence (3), anastomotic leak or dysfunction (6), other atresias (2), choledochal cyst (1), pyloric stenosis (1), and gastroesophageal reflux (2).(ABSTRACT TRUNCATED AT 250 WORDS)

Duodenal Obstruction↗

PubMed1987

A ten-year experience with the pediatric Kock pouch.

In 1981, a five-year experience with the pediatric Kock pouch was reported. This series has now increased to 20 patients with an overall experience of 10 years. There were 12 females and eight males ranging in age from 13 to 19 years. These pouches were all elective: 18 were converted from standard ileostomies, (six with proctectomy or Hartmann procedure), and two had a total proctocolectomy. Three of these conversions were for failed Swenson, Soave, and Duhamel procedures. Sixteen children had ulcerative colitis, three Hirschsprung's disease (one with colon atresia and imperforate anus with rectovaginal fistula), and one colonic polyposis (Gardner's syndrome). None had Crohn's disease. The 100% follow-up shows that 19 of the 20 teenagers have been followed for more than 1 year. The most important conclusions from this 10-year review of 20 teenage Kock pouch patients are (1) this continues to be a difficult operation with a significant potential for complications. (2) After 15 months, there is a very small incidence of nipple valve and outflow tract problems, but the possibility of pouchitis remains between 15 months and 5 years. (3) After 5 years, the chances of any kind of a problem with the Kock pouch are minimal. These children are all well, continent, happy, and back to a virtually normal life that includes marriage and pregnancy. The pediatric Kock pouch continues to be a reasonable alternative for the appropriate teenager, when a standard ileostomy is not desired and/or a pelvic pouch is impossible. It must be done by a surgeon with the necessary interest and experience. Long-term follow-up is essential.

Adolescent↗

PubMed1987

Successful management of congenital tracheal stenosis in infancy.

Seven infants with congenital tracheal stenosis were evaluated and treated to assess the efficacy of current techniques of management. All had multiple congenital anomalies in addition to tracheal stenosis. Notably, three infants had imperforate anus and three had vascular rings. Each infant presented early with respiratory distress. While bronchoscopy and/or bronchography were performed in some, the diagnosis could be made from high contrast x-rays of the chest in each instance. The complexity of the tracheal lesion(s) determined the operative approach. Complicating factors included tracheomalacia, multiple stenoses, pulmonary hypoplasia, and carinal involvement. The trachea was approached through an anterolateral thoracotomy in five cases and through the neck in one. Cardiopulmonary bypass was never required. Simple resection was possible only once. Four patients received costal cartilage grafts, and one graft was created from dura. Grafts varied from 2 1/2 to 6 cm in length and extended onto the bronchi in two cases. All patients with grafts were treated with postoperative endotracheal stents and ventilatory assistance and all were extubated successfully. One patient had trisomy 16p+ detected after successful extubation and died later. One patient was treated expectantly and died. While each case must be individualized, repair of complex tracheal stenosis often results in dramatic immediate improvement and long-term success.

Child, Preschool↗

PubMed1988

Double split of notochord with massive prolapse of the gut.

An unusual case of split notochord syndrome associated with double split of the spine, total prolapse of the gastrointestinal tract, short colon, and imperforate anus is reported. The absence of any neurologic deficit was suggestive of diplomyelia.

Abnormalities, Multiple↗

PubMed1989

Esophageal atresia with distal tracheoesophageal fistula: associated anomalies and prognosis in the 1980s.

Ninety-seven newborns with esophageal atresia and distal tracheoesophageal fistula (EA-TEF) were treated between 1979 and 1985 inclusive; there were 54 boys and 43 girls. Their weights ranged from 800 to 4,000 g (average, 2.5 kg). They included: 28 neonates with cardiac defects (most common: patent ductus arteriosus [PDA], ventricular septal defect and atrial septal defect [VSD-ASD]), of whom 18 survived (64%); 17 babies with other gastrointestinal anomalies (imperforate anus, duodenal atresia), of whom 12 survived (70%); 12 patients with skeletal malformations (digital, vertebral), of whom 11 survived (91%); 8 newborns with genitourinary abnormalities (hypospadias, undescended testis), of whom 6 survived (75%); and 16 infants with other congenital lesions (trisomy 18, lung agenesis-hypoplasia), of whom 3 survived (18%). Forty-six infants (average, 2.7 kg) had no other anomalies and all survived. As the number of systems with defects increased, both the weight of the baby and survival rate decreased. From this entire series of 97 newborns with EA-TEF, 81 (83%) survived (average, 2.3 kg). Sixteen babies died (average, 1.9 kg); 11 had defects incompatible with life. Eleven of the 16 were never operated on (seven patients with trisomy died within 5 days, and four patients with complex cardiac defects died within 3 weeks). Four of the 16 who were operated on died between 3 months and 2 years from chest problems and one newborn died in the operating room. We concluded that (1) newborns who have the common type of EA-TEF will almost certainly survive if there are no other anomalies; (2) the most frequently associated congenital defects are cardiovascular (28%), gastrointestinal (17%), skeletal (12%), and genitourinary (8%); (3) as the number of systems with defects increases, the weight of the baby and its survival rate fall; and (4) 11% will have trisomy and/or complex cardiac defects with no survival.

Abnormalities, Multiple↗

PubMed1989

Posterior sagittal anorectoplasty for pediatric recurrent rectal prolapse.

The recent use of the posterior sagittal anorectoplasty for repair of high imperforate anus has demonstrated several advantages: elimination of laparotomy, more direct approach, easier division of rectourethral fistula, more exact identification of the muscles of fecal continence, proper relocation of anorectum within these muscles and sphincters, and virtual elimination of postoperative anal prolapse. It is this latter advantage that attracted us to use this procedure for the repair of a recurrent rectal prolapse in a 1-year-old girl who also had a recurrent bladder exstrophy. The latter probably contributed to her constantly pushing out her rectum, which easily admitted two fingers. Two attempts were made to repair the rectal prolapse using the subcutaneous Thiersch's perianal technique; however, each was successful for only 6 weeks. When her recurrent bladder exstrophy was repaired, we also repaired her recurrent rectal prolapse using the posterior sagittal anorectoplasty. The midline sacrococcygeal incision was carried down to but not through the external sphincter, and the patulous rectum was plicated back to a normal size. Reapproximation of the levator sling and lower muscle complex then incorporated the plicated rectum. Both repairs remain intact after 1 year.

Anal Canal↗

PubMed1989

Malrotation of the intestines in children: the effect of age on presentation and therapy.

Because of the devastating consequences of midgut volvulus as a result of malrotation, we reviewed the charts of 70 consecutive children to define the spectrum of presentation. Although 27 patients (39%) had presenting symptoms within the first ten days of life, 35 (50%) were older than 2 months of age. In general, the older children had a longer course of vague, antecedent symptoms such as intermittent, nonbilious vomiting and chronic abdominal pain. Associated congenital anomalies were common, with 32 patients (46%) presenting with 56 anomalies, the most prevalent of which were intestinal atresia, imperforate anus, duodenal web, and cardiac and orthopedic anomalies. Upper gastrointestinal (GI) series revealed the diagnosis in 29 cases (41%), as did contrast enema in 24 (34%). It is important to note that volvulus, intestinal gangrene, and mortality occurred regardless of age or chronicity of symptoms. Fifteen patients (21%) were discovered serendipitously while being evaluated and treated for seemingly unrelated conditions. No morbidity of mortality occurred in those patients who underwent subsequent semielective Ladd's procedure. The majority of morbidity and all seven mortalities occurred in patients with volvulus and intestinal necrosis. This study emphasizes the need for consideration of Ladd's procedure for children of all ages. In addition, due to the broad range of initial symptoms, a high index of suspicion is required in evaluating children with possible malrotation. Because it remains impossible to predict which patients will have catastrophic complications (based on age or type of presentation), we urge that even incidentally discovered patients with intestinal malrotation undergo Ladd's procedure.

Abnormalities, Multiple↗

PubMed1996

Management of the gastrointestinal tract and nutrition in patients with cloacal exstrophy.

Cloacal exstrophy is a rare condition in which there is a complex set of congenital anomalies that affect multiple-organ systems, including the gastrointestinal tract. Twenty-six patients with cloacal exstrophy have been treated at the authors' institution during the last 20 years. Gastrointestinal features usually included omphalocele, exstrophy of an everted cecal plate, a short blind-ending distal colon, imperforate anus, and, occasionally, a shortened small bowel. Additional gastrointestinal anomalies included four cases of colonic duplication, one duodenal web, and one malrotation. The average time until the initiation of enteral feeding after initial surgery was 15.6 days, and the time until discontinuation of total parenteral nutrition (TPN) was 36 days. One patient with short bowel syndrome died of TPN-associated liver failure. Five other patients exhibited short bowel physiology, but ultimately each was weaned from supplemental intravenous hyperalimentation. Four patients have undergone posterior sagittal anorectoplasty, and one has had perineal anoplasty. Of these patients, two are continent and one is free of soilage on a bowel management program. In the authors' experience with management of the gastrointestinal tract and nutrition in patients born with cloacal exstrophy, many patients initially exhibited short bowel physiology, although most eventually adapted. However, very few patients have been able to achieve bowel control.

Abnormalities, Multiple↗

PubMed1997

Gluteus maximus augmentation for the treatment of fecal incontinence.

Fecal incontinence is a devastating problem for school-aged children and adults. Medical and biofeedback therapies are unsuccessful in most patients who have severely defective internal and external sphincters. Continued fecal incontinence frequently leads to social isolation and withdrawal. Gluteus maximus augmentation of the sphincter mechanism is one surgical method for treating fecal incontinence. The authors present their results with gluteus maximus augmentation of the anal sphincter and describe patient selection criteria. From 1992 through 1996, seven patients underwent gluteus maximus augmentation of the anal sphincter for fecal incontinence. Six of these patients were children 5 to 6 years of age who had major deficiencies of their anorectal sphincter demonstrated by manometry. One patient was a 56-year-old adult woman who had acquired idiopathic fecal incontinence. Four of the six children (67%) had imperforate anus and two had cloacal anomalies (33%). The augmentation was performed in three stages. A sigmoid-end colostomy with a Hartman's pouch was followed 1 month later by rotation of a portion of the gluteus maximus for anorectal sphincter augmentation. A colostomy take down was performed 2 to 4 months later. All patients underwent dilatation after sphincter augmentation and were taught muscle exercises for using their neosphincter during the period before colostomy take down. Four of six children and the adult are continent postoperatively (71%). Both patients who remain incontinent are unable to sense rectal distention clinically or on anal manometric analysis but have excellent voluntary sphincter tone. Fecal incontinence can be successfully treated with gluteus maximus augmentation in carefully selected patients. Patients unable to sense rectal distension are unlikely to benefit from this procedure. The presence of a rectal reservoir and a skin-lined anal canal also appear to be important in attaining fecal continence.

Buttocks↗

PubMed1998

Split notochord syndrome with prolapsed congenital colostomy.

A case of split notochord syndrome associated with a prolapsed colostomylike dorsal enteric opening, a foreshortened colon, imperforate anus, and meningocele is presented. The surgical management of this disorder is discussed and available literature is reviewed. The patient was successfully treated with a combined, single-stage surgical correction.

Abnormalities, Multiple↗