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Duodenal atresia and gastric antral web. A significant lesson to learn.

We report the case of a child who had a duodenal atresia and also a gastric-antral web. The two conditions are not known to be associated. The child had a duodenoduodenostomy for the duodenal atresia that had been diagnosed on a "double bubble" appearance at antenatal ultrasound scan. She eventually had a second laparotomy due to persistent gastric outlet obstruction, at which a pyloroplasty was performed and an antral web excised. The child remains well four months after discharge. This case has taught us several salutary lessons, which we consider relevant to share with colleagues.

Abnormalities, Multiple↗

Duodenal obstruction in Nigerian newborns and infants.

Experience with the management of congenital duodenal obstruction in a tropical environment is analysed with a view to highlighting some of the problems and suggesting solutions. 30 infants with duodenal obstruction were treated at the Lagos University Teaching Hospital between 1978 and 1985. 15 (50%) infants had duodenal atersia. 7 (23%) had Ladd's bands, 6 (20%) had duodenal diaphragm and there was one bay with partial duodenal obstruction caused by a duplication cyst. The associated abnormalities encountered in 7 infants are distal bowel atresia (3), partial situs inversus (3) and Trisomy 21 (1). Delay in presentation and inadequate facilities constituted the major constraints. 7 of the 25 infants that had corrective surgery died, a mortality of 28%. All the infants that were treated within the first 8 days of life survived. After this period survival was restricted to those with incomplete obstruction. Mortality can be reduced by the provision of adequate facilities and health education.

Duodenal Obstruction↗

[Cholestatic icterus during parenteral feeding in the newborn ano infants].

The authors observed six cases of cholestatis jaundice occuring during parenteral alimentation in the newborn and the infant. In 4 cases, local factors (duodenal atresia, necrosing enteritis of prematurity) or general factors (mucoviscidosis) probably played an essential role. In two cases, the parenteral alimentation seemed to be a main factor, but in one case with excessive and imbalanced supplies of amino acids. Three patients survived, with regression of the jaundice in 15 days to three weeks following the interruption of parenteral alimentation.

Amino Acids↗

The proximal mesenteric flap: a method for closing large mesenteric defects in jejunal atresia.

In selected patients with jejunal atresia, closure of the associated large mesenteric defect remains a technical problem. A case of jejunal atresia is reported in which a segment of dysfunctional proximal jejunum was resected and its mesentery retained, creating a flap of mesenteric tissue that was used to close a large defect. This technique allowed the closure of the mesenteric defect with a decreased chance of injury or kinking of the tenuous distal mesenteric vessels, improved orientation and stabilization of the distal bowel, and, theoretically, improved blood flow to the distal segment.

Female↗

Identical twins with malrotation and type IV jejunal atresia.

Identical twins are reported, who associated with maternal anaphylactic shock at 101/2 weeks gestation, acquired a type IV jejunal atresia with total absorption of the small bowel beyond the third portion of the duodenum and persistence of the most terminal length of ileum in the ileocolic artery in an apple peel configuration. Both infants survived a tapered jejunoileostomy and have undergone a successful transition to a regular diet. This accidental occurrence in two infants at the same time and in an identical fashion is a rare event.

Diseases in Twins↗

Gastric pneumatosis associated with preduodenal portal vein, duodenal atresia, and asplenia.

An 8-day-old newborn presented with non-bilious vomiting, upper abdominal fullness, and failure to pass meconium. Plain radiographs revealed gastric pneumatosis (GP). At operation, he was found to have a non-obstructive preduodenal portal vein, preampullary duodenal atresia, asplenia, and malrotation. The baby was treated by duodeno-duodenostomy without mobilizing the portal vein and correction of the malrotation according to Ladd's procedure. He made an uneventful recovery and the GP resolved spontaneously. The malformative process was believed to have occurred at or soon after the 5th week of gestation, and the GP probably resulted from intramural air tracking through mucosal tears caused by high intragastric pressure.

Cysts↗

Segmental atresia of the transverse colon in a foal with concurrent equine herpes virus-1 infection.

Segmental atresia of the transverse colon was observed at necropsy in a neonatal foal. The dorsal and ventral components of the large colon were fused, and ended blindly. The small colon was collapsed and completely closed at its cranial end. The right and left dorsal and ventral colons were fused into one blind-ended tube. Histologically, eosinophilic intranuclear inclusion bodies demonstrative of Equine Rhinopneumonitis were present in the thymus.

Animals↗

A case report of midgut atresia and spontaneous closure of gastroschisis.

We report a case in which a neonate with a prenatal diagnosis of gastroschisis was born with midgut atresia and the mummified remains of the midgut arising from a spontaneously closed abdominal wall defect. As our ability to prenatally diagnose abdominal wall defects has improved, we have gained some insight into the prenatal natural history of this pathological process. We present a case in which an abdominal wall defect spontaneously closed and was associated with an apparent in utero midgut vascular accident. This unusual case provides some insight into the mechanisms underlying the pathophysiology of gastroschisis.

Adolescent↗

Jejunal atresia with "apple peel" deformity: report of one case.

A case of jejunal atresia with "apple peel" deformity is presented. A 27-year-old woman, gravida 3, para 2, suffered from premature labor pain and vaginal bleeding at 32 weeks of gestation. Prenatal ultrasound examination revealed presence of three areas of cystic dilatation in the fetal abdomen and marked polyhydramnios, indicating a high bowel obstruction. A female baby was delivered by Cesarean section with Apgar scores of 7 and 8 at one and five minutes, respectively. Physical examination showed abdominal distention. Nasogastric tube aspiration got more than 50 ml of blood-tinged fluid. Roentgenography demonstrated a high jejunal obstruction as well as a normally rotated colon. Laparotomy disclosed proximal jejunal atresia with typical "apple peel" appearance of the distal small bowel. An end-to-end anastomosis was performed smoothly after resection of both the proximal and distal small bowels. A reduction tapering antimesenteric jejunoplasty was also done. Partial parenteral nutrition had been administered for 10 days followed by enteric feeding. The postoperative course was uneventful. Her chromosomal analysis revealed a normal karyotype of 46,XX.

Adult↗

Duodenojejunal atresia with absent dorsal mesentery, choledochal cyst, and malrotation in a premature newborn--a case report.

We present the case of a premature baby who was born with the following conditions: an extensive atresia from the first part of the duodenum to the mid small bowel; malrotation of the distal part, in volvulus and in an "apple peel" configuration; no connection of the bile ducts to the bowel; and presence of a type II choledochal cyst. To our knowledge, this is the first case in which a combination of these anomalies is reported. A brief review of the relevant literature is also presented.

Abnormalities, Multiple↗

[Treatment results following the pull-through operation and continence-improving operations in high anal and rectum atresia].

Of 174 patients with anorectal malformations, among them 71 children with high atresia, 59 patients could be followed-up. 57% were entirely incontinent according to electromanometrical findings. These patients necessarily needed sphincter-replacement operations to improve the function of the sphincter-apparatus. Eighty-one sphincter-replacement operations were performed in 68 patients, among them 56 gracilis transplantations, 12 palmaris-longus transplantations, and 4 stalked smooth muscle transplantations. All sphincter replacement operations improved continence considerably.

Adolescent↗

Radial ray defects, renal ectopia, duodenal atresia and hydrocephalus: the extended spectrum for Fanconi anaemia.

We present two families with a striking concordance of multiple anomalies. These include duodenal and other bowel atresia, radial ray defects especially absent or vestigial thumb, renal ectopia and hydrocephalus. The presence of diagnostically raised sensitivity to mitomycin C in the first family has confirmed Fanconi anaemia. This has further increased the spectrum of abnormalities in Fanconi anaemia and highlights the importance of mitomycin C analysis if elements of this spectrum are encountered.

Bone and Bones↗

Maternal ultrasonography for the antenatal diagnosis of surgically significant neonatal anomalies.

The increased use of ultrasonography in the management and evaluation of pregnancy has provided a unique opportunity to observe the anatomy of the developing fetus from 12 weeks gestation until term. Twenty-eight surgically important anatomic abnormalities have been diagnosed in utero by sonographic examinations at our affiliated institutions over the past three and a half years. These include ascites (five cases), gastroschisis (four cases), omphalocele (three cases), sacrococcygeal teratoma, cystic hygroma, hydrocele, duodenal atresia, multicystic kidney (two cases each), and one each of jejunal atresia, conjoined twins, ureteropelvic junction obstruction, urethral valves, urethral agenesis, and hydronephrosis secondary to reflux. Prenatal diagnosis by ultrasonographic examination has signifcantly improved perinatal management. Elective caesarean section has benefited infants with lesions causing dystocia, such as sacrococcygeal teratoma, omphalocele, and conjoined twins. Advance notification of surgeons and neonatalogists has reduced the delays of postnatal evaluation and treatment that contribute, significantly, to complications and death. In addition, transfer of the pregnant mother carrying an infant with a significant surgical anomaly to a center with facilities for neonatal surgery and specialized postoperative care can be properly planned for in advance. In the near future, intrauterine fetal surgery or palliative intervention may provide increased salvage of patients with obstructive uropathy and diaphragmatic hernia, both of which carry high mortality rates secondary to in utero damage. Sonography has proven useful in following the dilatation of either intestinal or urinary tract structures in utero. In our hands, maternal sonography has improved the surgical care of the newborn and may open a new frontier of intrauterine fetal surgery in the future.

Abdominal Muscles↗

[Presacral tumor with sacral meningocele].

Due to rectal atresia a 10-year-old boy had been operated on using Rehbein's procedure in babyhood in another hospital. Rectal incontinence remained and we planned to improve it by a gracilis plastic operation. During preoperative diagnosis we found a so-called "Currarino triad", because the boy did not only have an anorectal malformation but also an anterior sacral meningocele and a solid presacral tumour. Initially, two surgical interventions were done to remove the tumour and meningocele and three months later a gracilis plastic operation was performed successfully.

Child↗

The surgical repair of atresia ani in a Dobermann bitch.

The surgical repair of atresia ani accompanied by a large rectovaginal fistula was successfully performed in a 4-month old Dobermann bitch. As a young pup, the dog had been able to pass liquid stool through the vagina via a rectovaginal fistula, but the gradual change to a adult diet had resulted in considerable obstipation and tenesmus. The operation was performed experimentally, since the dog would have had to have been euthanased at this stage, and its success has allowed the dog to continue a satisfactory existence.

Anal Canal↗

Antenatal diagnosis of congenital anomalies.

Twelve neonates with surgical malformation diagnosed antenatally by ultrasound tomography (UST) with or without foetography were reported. Clinical diagnosis of these 12 cases were: Omphalocele 1, cervical teratoma 1, pyloric atresia 1, duodenal atresia 6, high jejunal atresia 2 and hydronephrosis 1. Three of these 12 deteriorated because of respiratory difficulty before the surgical intervention could be attempted. Operations were performed on the remaining 9 and 6 of them are surviving. The main causes of these postoperative deaths were respiratory complications and combined malformations. UST can detect numerous surgical malformations of the foetus. Antenatal diagnosis of the nature of a malformation may enable corrective surgery to be carried out with minor delay after birth, and good results can be obtained.

Congenital Abnormalities↗

Newborn duodenal atresia: an improving outlook.

From 1973 through 1983, 20 newborns with congenital duodenal atresia were treated. These patients are compared with our previous series and with other published series. There were no fatalities among the 19 patients who underwent operation, an improvement from the 72 percent survival rate in our previous series. Fifty-five percent of the patients had associated congenital anomalies, which frequently complicated their management. The use of a transanastomotic jejunal feeding tube resulted in delayed oral feeding and prolonged hospitalization, and cannot be recommended.

Abnormalities, Multiple↗

Colonic atresia: current results of treatment.

Eleven cases of colonic atresia presenting in a 13-year period are described. The incidence of this condition is estimated at 1 per 66,000 live births in the north-west of England. A higher incidence of involvement of the transverse colon has been noted in comparison with previously reported cases. Survival was unaffected by the type of surgical procedure, with both primary anastomosis after resection or staged reconstruction after initial colostomy giving excellent results. No deaths occurred in the ten patients subjected to surgical treatment.

Anastomosis, Surgical↗