Mesosigmoidoplasty.
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We describe here an autopsy case presenting bilateral cryptorchidism, left pelvic kidney and intestinal malrotation, evoking a "reverse rotation" with hypoplasia of the cecum. Furthermore, the colon showed alternate bands of stenosis and dilatation. This association has not yet been described, and is discussed in the light of the embryological events of kidney, gut and gonad development. Two hypotheses can explain gut stenoses (vascular consequences of the malrotation and type Hirschprung aganglionic segments).
Cecal pathology is commonly encountered and may represent a diagnostic challenge in patients with either acute or chronic clinical presentations. Although appendicitis accounts for the majority of surgical conditions presenting with right lower quadrant pain, readers should be aware of the broad spectrum of cecal pathologies and characteristic CT findings, which can be useful in establishing the correct diagnosis.
In right congenital diaphragmatic hernia (RCDH), several clinical diagnostic pitfalls are possible and should be known to those caring for infants and children with this disorder. The records of the 18 patients at Hotel Dieu de France Hospital with a history of CDH between 1990 and 1999 were collected; those of the ten who had a RCDH were reviewed retrospectively. The mean age at diagnosis was 6 months; the male-to-female ratio was 2:3. The delay between the first symptom and the diagnosis ranged between 0 and 10.5 months (mean 4.5 months). An acute presentation was observed in four cases, consisting of respiratory distress in three; the 4th presented with gastric volvulus and intestinal obstruction. The presenting symptoms were mild in four cases; recurrent respiratory infections in three and failure to thrive in one. The diagnosis was incidental in two cases during the evaluation of respiratory symptoms attributed to an atrial septal defect. The radiologic findings provided by a chest radiograph (CxR) were sufficient to make an accurate diagnosis in eight cases and peritoneography was useful in one. In six cases, the presenting CxR had been misinterpreted as normal or acute lobar pneumonia. Pathologic findings at surgery consisted of lateral and posterior right diaphragmatic defects in nine cases; the defect was lateral and anterior in one. A hernia sac was found in seven cases; malrotation was present in three. Surgical correction was done by an abdominal approach in nine cases and a thoracic approach in one. The diaphragmatic defect was repaired by transverse closure in six cases, diaphragm plication in three and prosthetic closure in one. The postoperative outcome was uneventful in eight cases. Two patients died. Thus, RCDH seems to cause less severe symptoms than left-sided LCDH. It usually manifests beyond the neonatal period as respiratory or gastrointestinal symptoms. The diagnosis should be made easily by a CxR. The presence of a hernia sac correlated with a mild presentation. An abdominal surgical approach is preferred.
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From 1972 to 1990, 69 cases of gastroschisis were treated at Akron Children's Hospital Medical Center. Eighty-one percent of these patients underwent primary closure of their abdominal wall defect. Thirteen of 69 patients (19%) required Silastic silos with final closure in an average of 7.8 days. There was no sex predilection, the average birth weight was 2,473 g, and the mean gestational age was 36.3 weeks. Twenty-six percent had associated anomalies, the majority were intestinal atresia, volvulus, and/or undescended testicles. Seventy-seven percent of the infants were delivered vaginally. Fourteen children were delivered via cesarean section. Seven cesarean sections were done solely for prenatal ultrasonic identification of an abdominal wall defect. There was no improvement in hospital stay, complications, days until enteral feeds were tolerated, days intubated, or number of surgical procedures in this group. In 14 patients, mesh sheeting (Marlex, Silastic) was used in the final closure. Sixty-four percent of these incurred wound breakdown necessitating removal of the mesh. This compares with a 3.2% wound breakdown in the nonmesh group. The average hospital stay was 43.9 days and the average time to enteral feeds 20.2 days. Sixty-four percent of the patients required postoperative intubation for an average of 5.5 days. The overall mortality rate was 4.3%. The present data do not support gastroschisis alone as an indication for cesarean section. The data indicate that mesh be avoided in the final closure if possible and support a favorable prognosis for most babies.
Hirschsprung's disease (HD) involves the entire colon in less than 5% of cases, and the association of extensive HD with intestinal malrotation is very rare. This association of symptoms may delay both diagnosis and treatment. An infant presented with an intermittent occlusive syndrome that began neonatally. Intestinal malrotation was diagnosed radiologically, and treated surgically when the child was 2 months old. However, a chronic occlusion persisted. Biopsies of the rectum and the appendix demonstrated an absence of neurons in intestinal plexi. When the child was 17 months old, ileostomy and surgical excision of the segment affected by HD (the colon and terminal ileum) were performed. An ileoanal anastomosis was performed at the age of 29 months, with favorable outcome. The persistence of symptoms of intestinal occlusion after attempted treatment of intestinal malrotation must therefore suggest the possibility of associated HD in a young child.
BACKGROUND: This study examined the effects of enterally administered epidermal growth factor (EGF) on nutrient absorption and tolerance of enteral feeds in pediatric patients with short bowel syndrome (SBS). METHODS: Patients identified with severe SBS (<25% bowel length predicted for age) were prospectively enrolled in treatment using human recombinant EGF (1-53); 100 microg/kg per day given mixed with enteral feeds and patients were treated for 6 weeks. End points followed were patient weight, tolerance of enteral feeds, nutrient absorption, and intestinal permeability as determined using carbohydrate probes and hematologic values for liver function parameters. RESULTS: Five patients were treated with EGF; all showed a significant improvement in carbohydrate absorption (3-0 methylglucose): absorption 24.7% +/- 9.7% pretreatment vs 34.1% +/- 13.8% posttreatment and improved tolerance of enteral feeds (enteral energy as % of total energy, 25% +/- 28% pretreatment vs 36% +/- 24% posttreatment; mean +/- SD; P < .05 by Wilcoxon's signed rank test). Epidermal growth factor treatment was not associated with significant changes in intestinal permeability, the rate of weight gain, or liver function tests. During the treatment phase, no patients developed episodes of sepsis; however, within 2 weeks of discontinuation of EGF treatment, 3 patients developed septic episodes. No adverse effects of EGF administration were noted. CONCLUSIONS: These results suggest that enteral treatment with EGF in pediatric SBS improves nutrient absorption, increases tolerance with enteral feeds, and may improve the infection rate. Further studies exploring treatment strategies including the timing and duration of EGF administration are indicated.
The informed consent process for emergency surgery in children poses a challenge for pediatric surgeons because the child and his/her surrogates (usually parents) must make medical decisions in a relatively short period. The unique circumstances of a surgical emergency create potential barriers to achieving the central goals of the informed consent process, respect for patient autonomy and beneficence. The purpose of this review is to provide a practical guide for pediatric surgeons on the informed consent process as it applies to emergency surgery in pediatric patients. We will also discuss innovative methods of preoperative education that can be adopted in the emergency setting and highlight areas in which further research might help to improve this important aspect of surgical care.
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Wandering spleen is an uncommon clinical entity, which rarely affects children and adolescents. It is usually described in adults, especially women of childbearing age. Discussion in the literature has been limited to case reports and small case series. Here the authors present 2 children and 1 adolescent that presented to the authors institution over a 9-month period with complications from a wandering spleen. All 3 patients had very different clinical presentations, and this variety is a constant theme throughout the literature. We also identified an additional 127 cases of wandering spleen in patients younger than 21 years. In this report we discuss the clinical presentations, diagnostic evaluation, treatment options, and clinical outcomes as they relate to wandering spleen.
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We report a clinical case of a persistent left superior vena cava discovered in a 50-year-old female patient when a port-a-cath was inserted. This already seldom malformation was associated with an arteria lusoria and polysplenia with left inferior vena cava with hemiazygos continuation, right-sided stomach, short pancreas, preduodenal portal vein and intestinal malrotation, but without any cardiac abnormalities.
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Intestinal obstruction in the newborn infant and older child may be due to a variety of conditions, including atresia and stenosis, annular pancreas, malrotation, duplication cyst, meconium ileus, meconium plug syndrome and neonatal small left colon syndrome, Hirschsprung's disease, neoplasia, trauma, and other rarer causes. The mode of presentation can be acute or more chronic with systemic upset due to shock. Neonates, more so than older children, with unrecognized intestinal obstruction deteriorate rapidly, show an increase of associated morbidity and mortality and appropriate surgical treatment becomes more hazardous. Early diagnosis depends largely on the prompt detection of obstructive manifestations by the clinician and the subsequent accurate interpretation of radiographic findings and other investigations, leading to definitive treatment, which should always be preceded by appropriate resuscitation/preparation of the infant/child. Management of intestinal obstruction will almost always be surgical, apart from some notable exceptions and all are discussed in more detail. With the advent of pediatric and neonatal intensive care and multidisciplinary care, the morbidity and mortality of cases of intestinal obstruction reported in current series is generally extremely low and mainly determined by the coexistence of other major congenital anomalies (eg, cardiac), delays in diagnosis and treatment or coexisting medical conditions. Newer treatments and future developments may reduce the residual mortality in such cases as ultrashort-bowel syndrome.