Biomedical subjects
Publications and source records attributed to Grover M Hutchins.
Purulent pericarditis is a cardiac emergency that can be difficult to diagnose and can be rapidly fatal. We report the case of a child with Down syndrome and recent atrial and ventricular septal defect repair who died from Haemophilus influenzae serotype f pericarditis.
We report on the autopsy findings of a 37-year-old man with a complex karyotype (mos46,XY,del(18)(p11.1)[14]/46,XY, -13, del (18)(p11.1), +20[8]/47,XY,del(18)(p11.1), +20[8]). He was known to be blind, non-ambulatory, have severe mental retardation, and a seizure disorder. External physical findings at the time of autopsy included micrognathia, short stubby fingers, and rocker bottom feet. Left lobe dominance of the liver and mislocation of the ileocecal junction and appendix were noted on internal examination. The brain was small (700 g) and poorly developed. Microscopically it showed an absence of neurons in the olivary and dentate nuclei, absence of Purkinje cells in the cerebellum, severe depletion of internal granular cells in the cerebellum, and cerebellar dysplasia. Fat infiltration was noted in an unusual distribution in several organs including a pattern in the heart consistent with arrythmogenic right ventricular dysplasia (ARVD). Findings of this mosaic chromosomal karyotype have not been previously described. This report will discuss this individuals physical findings and their relation to similar monochromosomal aberrations.
BACKGROUND: Staged palliative surgical procedures have been an effective treatment of complex congenital heart defects. The Fontan procedure has been of particular benefit to infants with functional single-ventricle complexes but with the consequence of a sustained increase of right-sided venous pressure. METHODS: We reviewed the clinical and pathologic features of 9 autopsied patients having undergone the Fontan procedure, with special attention given to their liver pathology. RESULTS: The 9 patients died from a few hours to 18 years after the Fontan operation. Chronic passive congestion was seen in 7 patients, and 4 patients surviving 4 to 18 years also had cardiac cirrhosis. Hepatic adenoma in the setting of cardiac cirrhosis was found in a patient surviving for 9 years. One patient surviving for 18 years had hepatocellular carcinoma superimposed on cardiac cirrhosis. Rupture of the hepatoma in this case led to fatal hemorrhage. CONCLUSION: The study shows that chronically increased hepatic venous pressure from the Fontan procedure might lead to chronic passive congestion, cardiac cirrhosis, hepatic adenoma, and hepatocellular carcinoma.
A 14-year-old adolescent girl presented with severe congestive heart failure, progressive throughout 3 months. A precordial thrill, machinery-like murmur, and right bundle branch block were noted. Death occurred despite digitalis and diuretic therapy and removal of pleural and ascitic fluid. The autopsy revealed 2 multilocular cystic structures in the interventricular septum consistent with being spontaneously drained valve ring abscesses. One of these lesions formed a fistulous communication that penetrated through the interventricular septum between the right aortic sinus of Valsalva and the crista supraventricularis that connected to the right ventricle. Another lesion, an adjacent separate but similar cystlike structure, communicated only with the left ventricular cavity. Although the cause of these lesions is uncertain, it seems probable that they are the residue of spontaneously drained and healed valve ring abscesses. Max Brödel, a medical illustrator and the first director of the Department of Art as Applied to Medicine at The Johns Hopkins University, drew previously unpublished figures of this patient's cardiac lesions. These illustrations exhibit Brödel's superb command of both art and medicine essential to his ability to make complex anatomic relationships demonstrable. We discuss Brödel's career and his influence on both the art and science of medicine.
William H. Welch, MD, and his colleagues performed an autopsy at The Johns Hopkins Hospital in October 1891 on a 38-year-old man and discovered a new bacterium, Bacillus aerogenes capsulatus. During the postmortem examination, gas bubbles were noted within many of the patient's blood vessels. Welch's laboratory personnel determined that a previously unknown bacterium was the source of the gas. Through a series of experiments, the organism's characteristics were described and its pathophysiology was detailed, findings that proved accurate in explaining gas gangrene during World War I. Welch never followed up these initial investigations with more experimentation. His subsequent writings regarding the bacterium that came to be known, appropriately, as Bacillus welchii consisted mostly of case reports from other medical institutions and summaries of previous data.
CONTEXT: Thrombotic thrombocytopenic purpura (TTP) and the hemolytic uremic syndrome (HUS) share many clinical features and have been difficult to separate into distinct entities. Histologic examination of organs from autopsied patients suggested that TTP and HUS have dissimilar lesions of different severity and distribution. OBJECTIVE: To perform a retrospective observational review of autopsied patients with TTP or HUS to compare the nature and severity of the lesions found. DESIGN: To examine the pathologic features of these conditions, we reviewed all cases among 51 350 indexed autopsies at The Johns Hopkins Hospital (Baltimore, Md) diagnosed with TTP or HUS, and included those showing multiple arteriolar thrombi or their sequela. RESULTS: The 56 cases that met the inclusion criteria fell into 2 distinct groups, based on distribution and severity of arteriolar lesions. In 25 patients classified as having TTP, platelet-rich thrombi were present-in decreasing severity-in heart, pancreas, kidney, adrenal gland, and brain. In 31 patients with HUS, fibrin/red cell-rich thrombi were present, largely confined to the kidney and often severe, and only 6 cases showed pancreas involvement, 4 adrenal gland involvement, 2 brain involvement, and 1 heart involvement. CONCLUSION: Despite similar clinical features and therapeutic approaches, TTP and HUS each have a characteristic constellation of histopathologic findings. This observation suggests that TTP and HUS are 2 distinct disease entities with different pathophysiologies, and that they do not represent a spectrum of the same disease process.
We report the case of a 23-year-old woman who was abused at the age of 5 months. She suffered from complications frequently associated with shaken baby syndrome, such as hydrocephalus secondary to subarachnoid hemorrhage. The patient underwent a procedure to place a ventriculoperitoneal shunt 3 weeks after her presentation with signs of abuse. The ventricular shunt remained in place throughout her life, and the patient received multiple revisions. She also was noted to have a markedly thickened calvarium on both radiographs and computed tomographic scan at 6 years old. She died following an episode of grand mal status epilepticus. An autopsy was performed and her skull was found to be thickened circumferentially. Histologic examination revealed increased cancellous space with normal trabecular bone. It is hypothesized that intracranial hypotension resulting from chronic ventricular shunting lead to her thickened calvarium, a condition previously reported as hyperostosis cranii ex vacuo. Dural changes seen microscopically corroborate this hypothesis.
A bovine collagen matrix is sometimes used as a delivery medium during direct intratumoral injection of a chemotherapeutic agent. The bovine collagen enhances the dose and duration of local drug delivery and limits systemic toxicity. Although this strategy is advocated as a means of easy and effective delivery of chemotherapeutic drugs, the associated risks are not well defined. We report the case of a 71-year-old man with hepatocellular carcinoma who underwent weekly intratumoral injections of cisplatin in a bovine collagen matrix. During the third injection, he suddenly and unexpectedly underwent cardiac arrest and died. An autopsy disclosed diffuse occlusion of the pulmonary microcirculation by bovine collagen. The collagen emboli were associated with an inflammatory infiltrate typical of bovine collagen-induced hypersensitivity. This case identifies a fatal complication of intratumoral chemotherapy injections using a bovine collagen matrix, which does not appear to have been previously reported. This case underscores the valuable role of the traditional autopsy examination as a means of identifying possible complications of novel oncologic strategies, which are being rapidly developed and implemented.
The pathogenesis of the prune belly syndrome (PBS) remains controversial, but two theories predominate. The first theory supports an obstructive phenomenon early in gestation leading to irreversible damage to the genitourinary tract and abdominal wall. The second theory suggests mesodermal injury between the 6th and 10th weeks of gestation as the primary abnormality. This paper reports of two fetuses with the PBS phenotype that were examined postmortem at our institution. Thorough examination of the lower urinary tract allowed demonstration of anatomic obstruction of the urethra in both cases. One case illustrated a relatively common pattern of proximal penile urethral obstruction, a flap-like obstruction between the prostatic and penile urethra. The other case provided what we believe to be the first description of PBS caused by severe phimosis.
BACKGROUND: Previous studies of the tracheoesophageal sulcus and the sulci of the developing heart have suggested that the catenoidal or saddle-shaped configuration of the sulcus had mechanical properties that were important to developmental processes by causing regional growth limitation. We examined the development of the human perineal region to determine if a similar configuration exists in relation to the urorectal septum. We wished to re-examine the controversial issue of the role of the urorectal sulcus in the partitioning of the cloaca. METHODS: Digitally scanned photomicrographs of serial histologic sections of embryos from Carnegie stages 13, 15, 18, and 22, obtained from the Carnegie Embryological Collection were used. Each image was digitally stacked, aligned, and isolated using image-editing software. Images were compiled using 3-D image-visualization software (T-Vox), into full 3-D voxel-based volume renderings. Similarly, digital models were made of the urogenital sinus, anorectum, cloaca, allantois, mesonephric ducts, ureters, and kidneys by isolating their associated epithelium in each histologic section and compiling the data in T-Vox. Methods were developed to create registration models for determining the exact position and orientation of outlined structures within the embryos. RESULTS: Models were oriented and resectioned to determine the configuration of the urorectal sulcus. The results show that the urorectal sulcus maintains a catenoidal configuration during the developmental period studied and, thus, would be expected to limit caudal growth of the urorectal septum. CONCLUSION: The observations support the concept that the urorectal septum is a passive structure that does not actively divide the cloaca into urogenital and anorectal components.
BACKGROUND/PURPOSE: The rationale for in utero repair of myelomeningocele has been supported experimentally by the observation of preserved neural function after prenatal closure of surgically created defects compared with nonrepaired controls. The mechanism of injury to the exposed neural elements is unknown. Postulated mechanisms include trauma to the herniated neural elements or progressive injury from amniotic fluid exposure as gestation proceeds. A component of amniotic fluid that may contribute to neural injury is meconium. In the current study the effect of human meconium on the exposed spinal cord in a fetal rat model of myelomeningocele was examined. METHODS: Twenty time-dated pregnant rats underwent laparotomy at 181/2 days of gestation. The exposed uterus was bathed in ritrodrine for tocolysis. The amniotic cavity was opened over the dorsal midline of the fetal rat, and, under a dissecting microscope (x25), a 2- to 3-level laminectomy was performed. Under magnification (x40), the translucent dura was opened using a 25-gauge needle as a knife. Two fetuses per dam were operated on. In the control group, the amniotic fluid was restored with saline solution, whereas in the experimental group a solution of Human meconium diluted (10%) in saline was used to restore the amniotic fluid. Fetuses were harvested by cesarean section at 211/2 days' gestational age. The liveborn pups were then killed and fixed in 10% formaline. Sections 10 micrometer thick were stained with H&E and studied by light microscopy for evidence of spinal cord injury. RESULTS: Seven of 20 (35%) experimental rat pups and 6 of 20 (30%) control rat pups were liveborn. All liveborn pups had severe paralysis of the hindlimbs and tail, so that functional differences between the 2 groups could not be detected. Histologic examination of 13 spinal cords at the site of surgical exposure showed that necrosis of neural tissue in 5 of 7 meconium-exposed rat pups was increased when compared with that observed in the 6 fetuses exposed to amniotic fluid without meconium. In general, inflammation was greater and repair processes appeared delayed in meconium-exposed rat pups. CONCLUSIONS: Exposure of the spinal cord of fetal rats to amniotic fluid by surgically created myelomeningocele leads to severe functional impairment. Histologically recognizable necrosis of neural elements was increased in those animals that were exposed to diluted human meconium in the amniotic fluid. The results support the hypothesis that meconium may contribute to the pathophysiology of spinal cord injury observed in myelomeningocele.
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Children with cerebral palsy (CP) develop difficulties with swallowing and bowel motility, although the underlying etiology of these disorders is unclear. The authors identified three children treated at their institution in the past 6 years who developed severe gastrointestinal dysfunction leading to gastric rupture after orthopaedic surgery; all three had a history that included CP (subtype spastic quadriparesis), low cognitive function, and gastrointestinal motility disorders. All three cases were fatal; in two patients the diagnosis was made at autopsy. No case of postoperative gastric rupture has been identified in children undergoing orthopaedic surgery without concomitant CP at the authors' institution. In this case series, they describe the preoperative and postoperative course of these three children. Since the only cure for gastric rupture is prompt surgical attention, orthopaedists should consider gastric rupture in the differential diagnosis of a postsurgical CP patient who becomes acutely unstable.
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