Malrotation of bowel: variable patterns with different surgical considerations.

The records of 53 patients with varied roentgenographic patterns of malrotation were reviewed. The patterns were categorized as A-Infantile (Al), A-Adult (Aa), B, C, and D. The differences in form were obvious and each differed clinically, radiographically, and surgically. The differences allowed the development of prognostic insights simply based on the presence and/or position of the ligament of Treitz and the cecum as shown by barium gastrointestinal examination. The literature was reviewed to find supportive or contradictory case material or other data that complemented or devalued the developed system for determining prognosis of patients with varied bowel patterns. The developed prognostic scheme seems to satisfy the requirements of reliability.

Shard-like calcifications in retroperitoneal teratomas.

Retroperitoneal teratomas can have characteristic roentgenographic findings. Three children with that neoplasm had roentgenographic assessments of the tumor which revealed similar "shard-like" calcifications. The effect of the tumor on the kidneys produced hypertension in all the patients which abated post-operatively. Extensive imaging evaluations in similar patients seems unnecessary since the findings are virtually pathognomonic. Only a single urinary tract study and plain film investigation of the abdomen are advised prior to expeditious surgery.

Intrathoracic desmoid tumor in a child.

The case history of a child with an intrathoracic desmoid tumor is presented. The insidious nature of the tumor's development and its possible relationship to previous surgery are noted. Desmoid tumors are rare in the childhood population and an intrathoracic site as the origin of one has not been reported previously.

Multiple endocrine neoplasia (MEN) syndrome type IIB: gastrointestinal manifestations.

Multiple endocrine neoplasia syndrome IIB is an inherited autosomal dominant disorder with variable penetrance. Multiple organ systems are involved with a characteristic triad of medullary thyroid carcinoma, pheochromocytoma, and alimentary tract ganglioneuromas. Most patients have characteristic facies with patulous lips and thickened tarsal plates of the eyelids. The entire gastrointestinal tract can be involved from tongue to anus. Tongue nodules are common. Altered intestinal motility, dilation, diverticula, and mucosal abnormality may be seen radiographically. Gastrointestinal symptoms are common, may be the presenting manifestation of the syndrome early in life, and allow the radiologist to suggest the proper diagnosis. Five patients are described. All had megacolon.

Eight-minute excretory urographic film: once is enough.

A film reading exercise was developed to test the advisability and accuracy of using one "postinfusion" roentgenogram instead of a series of films for a complete antegrade urographic evaluation. The experience of the radiologist making the interpretations was also considered. In most cases a singly eight-minute postinfusion delayed film showed enough detail to rule out pathology when none existed and adequately revealed either specific abnormal findings or suggested an abnormality existed when such was indeed present. No false negative call was made by and of the radiologists, but experience did aid in detailing the pathology.

Esophageal dysmotility and the sudden infant death syndrome: clinical experience.

Symptoms equated with "threatened" or "near-miss" sudden infant death syndrome (SIDS) were noted during feedings in 4 infants. In each case, barium esophagrams revealed dysmotility. Acute "near-miss" symptoms required cardiopulmonary resuscitation during esophagography in 2 infants. The authors suggest that esophageal dysmotility is a cause of "near-miss" SIDS and most likely is responsible for more fatalities than commonly realized. The cause-and-effect relationship is explained by a vagovagal reaction. Etiologies of esophageal dysmotility and possible prophylactic measures are discussed.

Esophageal dysmotility and the sudden infant death syndrome: experimental observations of neonatal puppies.

The authors review the relationship between esophageal dysfunction (dysmotility) and the unusual cardiopulmonary and neurological manifestations equated with the symptoms of the "near-miss" sudden infant death syndrome (SIDS). Six beagle puppies were studied to see whether esophageal irritation and dilatation could provoke any cardiac dysrhythmia. Some of the cardiographic irregularities observed in 10 of 11 trials occasionally precede fatal ventricular dysfunction. The authors conclude that esophageal irritation can be responsible for cardiac arrest and that one important cause of SIDS is esophageal dysmotility.

Malrotation simulating intussusception on barium enema.

Two cases of midgut malrotation are presented. Each had a clinical picture suggesting a possible intussusception. The radiographs also demonstrated findings suggestive of intussusception though these were subtle findings which could have suggested the correct diagnosis of malrotation.

Female epispadias: report of a case and review of the literature.

Female epispadias is a congenital anomaly representing a mild form of the spectrum of exstrophy of the bladder. Unrecognized an untreated, the handicap associated with the physical malformation can create overwhelming clinical and psychological problems for the affected female. Radiographic findings of spinal dysrhaphism with diastasis of the public bones are often associated with, and can afford the first clue to, the physical findings of absent clitoris, ununited labia and patulous and foreshortned urethra.

Benign neoplasmia and pseudoneoplasia of the small bowel in children.

Fourteen cases of benign tumorous conditions in children are reported. Each new growth described represents a true neoplasm, one simulating a true neoplasm, or one simulating a true tumor, clinically and/or radiographically. The series contains 10 female and 4 male patients. Most patients were under 1 year of age when manifestations--most often, gastrointestinal hemorrhage or bowel obstruction-- of the condition became evident. Nine patients had roentgenographic findings which indicated the presence of an abnormality, but the specificity of the findings was low. Some patients had cutaneous or other somatic clinical stigmata found in known syndromes also containing gastrointestinal pathology. These stigmata and the awareness of the clinical and roentgenographic manifestations of small bowel tumors in children should lead to an early diagnosis and hopefully to the usually successful surgical therapy.

Clinical and radiographic considerations of sacrococcygeal teratomas: an analysis of 26 new cases and review of the literature.

Teratomas develop most frequently in the sacrococcygeum and are often diagnosed by simple observation. Most sacrococcygeal teratomas are benign, and evident in the newborn. Females are affected four times more often than males, but affected males are more likely to have malignant tumors. Benign teratomas are generally noninvasive, cystic, and contain calcifications. Surgery is the primary, usually curative treatment, although malignant tumors can be treated effectively only by early diagnosis and removal. Twenty-six cases of sacrococcygeal teratoma were reviewed.

Vertebral malformations and associated somaticovisceral abnormalities.

Numerous references to spine abnormalities and associated somatic and/or visceral abnormalities are reported. An implication is present in each that the association is more than just coincidental and some cause and effect relationship might exist. A series of cases was collected in which congenital spine abnormalities were noted on roentgenograms from patients evaluated for varied reasons. Other roentgenograms of these patients and their medical records were reviewed in an attempt to find associated congenital abnormalities. Another group of patients with known congenital visceral malformations had their roentgenograms reviewed to see if they had spinal variations. It was found that patients with congenital vertebral malformations had an extremely high rate of associated visceral abnormalities, especially of the heart and kidneys. Other relationships between the spine and viscera include congenital lesions of the gastrointestinal and respiratory system and other parts of the renal system. This study also suggests the possible relationship between vesicoureteral reflux and spina bifida occulta. Embryologic considerations yield likely reasons for the relationships which have been suggested previously and are emphasised and augmented by this investigation. The supporting argument that a definite relationship exists between spinal variations and visceral abnormalities, as well as the embryologic reasons, is presented in this review of our selected cases and the appropriate literature.