Malrotation in patients with duodenal atresia: A true association or an expected finding on postoperative upper gastrointestinal barium study?

We retrospectively reviewed the imaging and surgical findings in 17 patients with duodenal atresia to determine (a) the frequency of coexistent malrotation in patients with duodenal atresia and (b) the reliability of the upper gastrointestinal barium study (UGI) in differentiating malrotation from postoperative deformity of the duodenal sweep after repair of duodenal atresia. Postoperatively, 9 (53%) of the 17 patients had UGI findings consistent with malrotation. Of these nine, only two had malrotation coexistent with duodenal atresia, while the other seven had normal midgut rotation demonstrated intraoperatively. The radiographic appearance of malrotation was simulated in two patients in whom the ligament of Treitz had been surgically divided, in three in whom the ligament had not been taken down, and in two in whom the status of the ligament was not specified in the surgical report. Although there is an association between duodenal atresia and malrotation, this cannot be accurately documented on postoperative UGI examination. Malrotation cannot be detected preoperatively because contrast material cannot pass beyond the level of the atresia. Postoperatively, surgical deformity of the duodenal sweep cannot be reliably distinguished from malrotation.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/46705/1/247_2005_Article_BF02012180.pd

Synchronous bilateral Wilm's tumor in the adolescent and adult

Bilateral Wilms' tumor in the adolescent and adult is extremely rare, with only four previous cases reported. Theories as to the etiology of this entity and a review of the demographics, diagnosis, staging, and treatment of Wilms' tumor with emphasis on bilateral disease and the older patient are presented. Current approaches to the patient with an atypical presentation of Wilms' tumor can result in excellent results similar to those seen with the more typical presentation.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47158/1/383_2004_Article_BF00185144.pd

Idiopathic gastric infarction in childhood

The case of a 6-year-old female with gastric infarction is presented. Despite a careful evaluation, no etiology could be determined in this child or, retrospectively, in several similar children described by other authors in the past. Vascular compromise was present although it is not possible to know whether this was a primary or a secondary event. It is important to recognize that gastric infarction can occur in children without obvious explanation.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47178/1/383_2004_Article_BF00182780.pd

Suction rectal biopsy in the diagnosis of Hirschsprung's disease and chronic constipation

Suction rectal biopsy has gained increased acceptance as the means of definitively diagnosing Hirschsprung's disease as well as excluding this diagnosis when evaluating the child with chronic constipation. During the 11-year period from 1 July 1974 through 30 June 1985 at the University of Michigan, Mott Children's Hospital, 309 suction rectal biopsy specimens were evaluated. Of these, 293 were done for the evaluation of chronic constipation and/or Hirschsprung's disease. The remaining 16 were performed as a part of the work-up in patients with neuromuscular, glycogen storage, inflammatory bowel, or other diseases. Forty-two (14%) of the 293 patients were diagnosed as having Hirschsprung's disease at an average age of 14.4 months. This diagnosis was ruled out in the remaining 251 patients, whose age at biopsy averaged 2.7 years. There was one false-negative reading representing an incidence of 0.3%, with no false-positives. There were no complications. The suction rectal biopsy is a bedside or clinic procedure that reliably provides pathologic material adequate for the accurate diagnosis or exclusion of Hirschsprung's disease and offers a number of advantages over manometric, radiographic, histochemical, and open, full-thickness biopsy techniques.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47146/1/383_2004_Article_BF00166866.pd

Hirschsprung's disease in adolescents and adults

Hirschsprung's disease in the adolescent and adult is a rare and often misdiagnosed cause of lifelong refractory constipation. Two adolescent and three adult patients with Hirschsprung's disease treated between 1973 and 1987 at the University of Michigan Medical Center are reported. Each patient presented with chronic constipation requiring enemas, cathartics, and multiple hospital admissions for management. Diagnosis in each case was made with barium enema and full-thickness rectal biopsy. Four patients underwent endorectal pull-through procedures, all with good long-term results. The fifth patient, initially treated with a Duhamel retrorectal pull-through procedure, required reoperation for constipation secondary to a retained rectal septum. Review of 199 cases of adult Hirschsprung's disease enables comparison of the various operative procedures for this disorder with respect to postoperative complications and functional outcomes. Anorectal myectomy with low anterior resection, the Duhamel-Martin procedure, and the Soave endorectal pull-through procedure are the most acceptable methods for surgical management .Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/41392/1/10350_2005_Article_BF02052222.pd

Trauma scores, accident deformity codes, and car restraints in children

The importance and effectiveness of the appropriate use of automobile restraints by young children has been emphasized in several studies. Once the child has entered the emergency care system, however, restraint use may not be the best predictor of injury severity. This study was undertaken to investigate the relationship of restraint status to morbidity and mortality in children examined in a hospital emergency facility following involvment in a motor vehicle crash (MVC). The emergency room charts of 101 children under 18 years of age, who were victims of MVCs, were reviewed and the following trauma scores were calculated: Glascow Coma Scale, Pediatric Trauma Score, Revised Trauma Score, Injury Severity Score, and Maximum Abbreviated Injury Score. In addition, the Traffic Accident Damage (TAD) score, an estimate of crash severity determined by the police at the accident scene, was recorded. The patients were age stratified as follows: 0 to 4 years (n = 24), 5 to 11 years (n = 29), and 12 to 17 years (n = 48). Fifty patients were appropriately restrained at the time of the crash. There was a significant correlation between mean trauma scores and mean TAD codes (P P > .05) across all age groups, and these children were grouped together as "unrestrained" in further analyses. In the 0 to 4 age group, there were no significant differences in mean trauma scores between restrained and improperly restrained or unrestrained children in contrast to the 5 to 11 and the 12 to 17 age groups. There were no significant difference between the distribution of restrained and unrestrained victims with regard to mean TAD scores (P = .5224) in the 0 to 4 age group. This study demonstrates a close correlation between mean trauma scores and vehicle deformity in all age groups, and shows that mean trauma scores appear to be independent of restraint use for the 0 to 4-year-old age subset. Therefore, a police-assigned crash severity score, such as the TAD, may be useful in the initial traige of pediatric trauma victims to an appropriate hospital or trauma center.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/31099/1/0000777.pd

Resolution of splenic injury after nonoperative management

Numerous studies have demonstrated success with nonoperative management of splenic injuries in pediatric patients. However, the resolution of the splenic injury has not been previously evaluated. The records of 50 pediatric patients with splenic injuries from blunt trauma treated nonoperatively between 1984 to 1992 were reviewed retrospectively. Abdominal computed tomography (CT) was performed at the time of injury and 6 weeks postinjury in 25 patients. These scans were reviewed and categorized by a modification of a previously reported grading system for parenchymal injury. All patients had healing of the splenic injuries, with complete resolution of the healing process observed at 6 weeks postinjury in 44%. Even those with shattered spleens (n = 6) had consistent improvement in splenic architecture, with resolution of fractures and/or contusions and return of splenic perfusion. Ten (77%) of 13 grade 1 and 2 injuries were completely resolved by the 6-week follow-up examination, whereas only one (8%) of 12 grade 3 to 5 injuries showed radiological resolution of splenic injuries. None of the 25 follow-up CT scans affected clinical decision-making or led to a deviation from the established protocol, which included a 3-month period of reduced activity. All 50 patients did well, without evidence of morbidity, mortality, or complications after return to full activity 3 months postinjury. These CT scan results and clinical data show (1) radiological resolution of splenic injury, with return toward a normal appearance by 6 weeks postinjury, (2) uniform success of nonoperative management of splenic injury in children, (3) that follow-up CT scans may be used to indicate earlier return to full activity in most cases of grade 1 and 2 splenic injuries, but otherwise they do not appear to contribute to the clinical management of the child after nonoperative treatment of splenic trauma.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/31314/1/0000223.pd

Surgical management of ambiguous genitalia in the infant and child

Proper gender assignment to a neonate born with ambiguous genitalia is a social emergency of the newborn period. Once an appropriate sex assignment has been made, the next critical step is performance, if needed, of a reconstructive procedure in a timely fashion. In an attempt to evaluate our experience with this unique group of patients, we have retrospectively reviewed the course of 69 children with ambiguous genitalia managed surgically at this institution between 1974 and 1989. This series consists of 32 genotypic females with congenital adrenal hyperplasia (CAH), 10 children with mixed gonadal dysgenesis (MGD), 10 male pseudohermaphrodites, 3 true hermaphrodites, 8 genotypic females with urogenital sinus anomalies (UGS), and 6 genotypic males with bilateral undescended testes and penoscrotal hypospadius. All newborns initially seen at this institution received proper sex assignment within the first week of life. Prior to 1980, 17 of the children with CAH underwent clitorectomy and vaginoplasty and three underwent clitoral recession and vaginoplasty. After 1980, 10 patients with CAH were managed with clitoral recession and vaginoplasty and 2 with vaginoplasty alone. Eight of 10 cases of MGD were given a female sex assignment and all 8 underwent gonadectomy due to the high risk of gonadoblastoma; the other 2 children were raised as males. There were 3 true hermaphrodites of which 2 were assigned female gender roles and were managed with a clitoral recession and vaginoplasty. All 10 male pseudohermaphrodites were raised as females and all underwent bilateral orchidectomy. The 8 children with UGS were raised as females and underwent vaginal reconstruction. Six genotypic males with penoscrotal hypospadius and bilateral undescended testes were raised as males and underwent hypospadius repair and bilateral orchidopexy. This retrospective review underscores the complexities of assessment and management of ambiguous genitalia in infants and children. It also reconfirms the data from other series showing that the vast majority of infants born with the four most common forms of ambiguous genitalia (CAH, MGD, male pseudoher-maphroditism, and true hermaphroditism) must be raised as females because of inadequacy of the phallus.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/29268/1/0000327.pd

Body fluid compartment changes following cardiopulmonary bypass in dogs

To investigate whether pulsatile perfusion affects postoperative edema, we examined weight, total body water (TBW), and extracellular fluid (ECF) following cardiopulmonary bypass (CPB) in dogs using three different perfusion systems. Fourteen dogs were divided into three groups differing only in the perfusion system used: Sarns nonpulsatile and pulsatile roller pumps and a University of Texas pulsatile pump. Weight, TBW, and ECF were measured preoperatively and on postoperative days (POD) 1, 3, and 7. No significant differences were seen in body weight, TBW, or ECF between groups. Body TBW (percent of body weight) rose significantly ( P =0.005) to 72% on POD 3 and 7. ECF (percent of body weight) rose to 58% by POD 7 ( P =0.008). These three perfusion systems produced no differences in the pattern of postoperative body fluid distribution after CPB, suggesting that there is no advantage to pulsatile perfusion for the purpose of decreasing postoperative edema.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47180/1/383_2004_Article_BF00189175.pd

Hirschsprung's disease in the newborn

The records of all patients with Hirschsprung's disease diagnosed and treated at our institution between 1 July 1974 and 31 August 1985 were reviewed. Of these 99 patients, 35 (35%) presented and were diagnosed within the first 30 days of life and constitute the basis of the present study group. Twenty-two infants (63%) had standard rectosigmoid disease with a male-to-female ratio of 2.2:1. Only one infant was premature. The spectrum of presenting signs included abdominal distension in 19 (54%), failure to pass meconium within the first 48 h of life in 16 (46%), “constipation” in 12 (34%), and vomiting in 9 (26%). Intestinal perforation was a presenting sign in 2 patients (6%) and enterocolitis occurred preoperatively in 4 (12%). Evaluation was facilitated by diagnostic barium enema in 60% of the patients. In those infants able to undergo elective evaluation, the definitive diagnosis was made by suction rectal biopsy, which was accurate in all cases. In addition to the high proportion of patients with long-segment disease (13 patients, 37%), there was a significant incidence of associated congenital anomalies (26%), including Down's syndrome in 5 (14%). Thirty-three of the 35 patients have undergone definitive treatment using the endorectal pull-through procedure, performed at an average age of 12 months, with no mortality related to the operation. In addition to highlighting the high incidence of congenital anomalies, the large proportion of neonates with long-segment disease, and the reliability of the diagnostic barium enema, this subgroup of patients with Hirschsprung's disease emphasizes the special diagnostic and management considerations required in the newborn infant who presents with sepsis of unknown etiology, intestinal obstruction, or constipation. A high index of suspicion, liberal use of suction rectal biopsy, early leveling colostomy, and definitive treatment by endorectal pull-through are important in achieving 0% operative mortality and excellent functional results.Peer Reviewedhttp://deepblue.lib.umich.edu/bitstream/2027.42/47145/1/383_2004_Article_BF00166865.pd