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Biomedical subjects

W H Hendren

Publications and source records attributed to W H Hendren.

At least 19 recordsLinked to original sources
PubMed1996

Congenital posterior urethral perineal fistulae: a unique form of urethral duplication.

Three unique cases of urethral duplication, often referred to as congenital posterior urethral perineal fistulae, are presented. Presenting signs and symptoms, evaluation, and treatment are discussed, and the literature is reviewed. Although labeled as a fistula in the literature, this entity most likely represents a distinct type of urethral duplication. A functional dorsal urethra separates it from the other forms of urethral duplication.

Abnormalities, Multiple↗
PubMed1995

Repair of the high vagina in girls with severely masculinized anatomy from the adrenogenital syndrome.

In the most severe form of congenital adrenal hyperplasia in females, the vagina is located above the level of the external urethral sphincter. Sixteen of these patients had reconstruction with a pull-through technique described previously. Nine are now adults; six have coitus and three do not. Five are young and sexually immature. Two have been lost to follow-up.

Adrenal Hyperplasia, Congenital↗
PubMed1995

Interposed jejunal segment with nipple valve to prevent reflux in biliary reconstruction.

BACKGROUND: Resection is the accepted management of a choledochal cyst. However, the debate continues regarding the optimal method of biliary reconstruction. The Roux-en-Y limb is used most frequently, but concerns have been raised about this method due to associated peptic ulcer disease, cholangitis, and poor growth. A method of reconstruction using an interposed segment of jejunum with a nipple valve placed between the common bile duct and the duodenum has been proposed. STUDY DESIGN: We have reviewed a series of 12 children requiring biliary reconstruction for choledochal cyst (11 children) and biliary stricture (one child). All had reconstruction with a nipple valve, and ten had an interposed segment of jejunum. RESULTS: All of the children are alive and have had follow-up evaluation from six months to 8.5 years (median of three years). Sequential examinations with ultrasound and biliary excretion scans have shown no evidence of obstruction, and liver function tests have remained normal. Three children have had cholangitis. One child had a brief episode in the perioperative period. The second child had cholangitis 16 months postoperatively, and the third child had multiple episodes of cholangitis. These latter two children were unique. One had Alonso-Lej type IV choledochal cyst with intrahepatic dilatation, which persisted after reconstruction. The other had a prior diversion with a Roux-en-Y limb from the gallbladder after resection of a choledochal cyst and had multiple episodes of cholangitis before reconstruction. These episodes are now controlled with chronic antibiotic suppression. Postoperative complications were limited to two episodes of obstruction of the small bowel requiring lysis of adhesions. No child has had peptic ulcer disease. These children have grown well after reconstruction, except for three with multiple anomalies or chronic pancreatitis. CONCLUSIONS: Biliary reconstruction with a jejunal interposition containing a nipple valve can be performed safely with a low incidence of complications. It offers a more physiologic method of reconstruction and a low incidence of postoperative cholangitis.

Adolescent↗
PubMed1994

Antenatal diagnosis of isolated congenital diaphragmatic hernia is not an indicator of outcome.

It has been reported previously that infants diagnosed with congenital diaphragmatic hernia (CDH) antenatally have a much poorer prognosis than those diagnosed postnatally. The authors identified 173 high-risk (symptomatic within the first 6 hours of life) infants with CDH treated in the past decade. Seventy-seven cases were diagnosed antenatally and 96 were diagnosed postnatally. The survival rate was slightly worse for the antenatal group (34% v 48% for the postnatal group; P = .04). However, 59 of the 173 patients (34%) had other life-threatening congenital anomalies. Among the 114 patients with isolated CDH, the survival rate increased to 59% in the antenatal group and 63% in the postnatal group--a difference that was not significant. The timing of antenatal diagnosis (> or < 25 weeks) had no impact on the statistics (P = .08). The only parameter consistently different between all groups and subgroups was the Apgar score at 5 minutes, which was always better in the antenatal group (P < .02), possibly reflecting more efficient resuscitation. Of the 59 patients with other life-threatening anomalies (42 cardiac), there was one survivor among the 34 in the antenatal group and only two among the 25 in the postnatal group, despite the fact that 39 patients had undergone resuscitation, which included ECMO in 25.(ABSTRACT TRUNCATED AT 250 WORDS)

Abnormalities, Multiple↗
PubMed1994

Use of bowel for vaginal reconstruction.

There is a wide range of congenital vaginal abnormalities that can be reconstructed using bowel. From 1964 to 1993 we used bowel for vaginal reconstruction in 65 patients and all but 5 were treated since 1981. Patient age ranged from 9 months to 26 years. In 29 patients the bowel was used to extend a short vagina to the perineum, and in 36 the entire vagina was absent and required a substitute vagina. Indications for surgery included cloacal malformations in 34 cases, congenital absence of vagina in 12, cloacal exstrophy in 9 genetic male subjects, postoperative exenteration in 7, intersex in 2 and bladder exstrophy in 1. Rectum was used in 12 cases, sigmoid colon in 27 and small bowel in 26. Ten patients who are now adults have reported satisfactory coitus.

Adolescent↗
PubMed1994

Long-term nutritional and metabolic consequences of pancreaticoduodenectomy in children.

BACKGROUND: The long-term nutritional and metabolic consequences of pancreaticoduodenectomy in children are unknown. METHODS: Five children were evaluated in a clinical research center 2.5 to 10 years after pancreaticoduodenectomy to assess their nutritional status based on patterns of growth and to assess their gastrointestinal function. Investigation included vitamin levels, a bentiromide study, and serum immunoreactive trypsinogen levels to evaluate pancreatic function and a d-xylose absorption and a radionuclide gastric emptying scan for intestinal absorption and motility. RESULTS: Children were able to grow after pancreaticoduodenectomy. Three remained in low percentile groups for height/weight ratio, and two were near or above normal. Low normal levels of the fat-soluble vitamins were present. Very low levels of pancreatic function were found based on the bentiromide and trypsinogen studies, whereas intestinal absorption of d-xylose was normal except for one patient with extremely rapid gastric emptying. CONCLUSIONS: After pancreaticoduodenectomy children can grow and develop normally if given adequate levels of oral pancreatic supplements to replace the severely decreased level of endogenous pancreatic enzymes after operation. Routine supplementation of the fat-soluble vitamins should be considered.

4-Aminobenzoic Acid↗
PubMed1993

Abnormal aorta and iliac arteries in children with urogenital abnormalities.

Retroiliac ureters have been found in association with a variety of urogenital abnormalities but the iliac artery usually retains a retroperitoneal position. We report 7 children with various urogenital abnormalities (cloacal malformation 4, bladder agenesis 1, urogenital sinus 1, neuropathic bladder 1) in whom the lower aorta and iliac arteries were so distorted as to pose a significant operative hazard. In all cases the distal aorta and/or iliac arteries lay within the peritoneal cavity, often anterior to the bladder. In 3 cases the aorta was fixed by a short ligament to the umbilicus, and in 3 patients one of the iliac arteries ran across the pelvis behind the pubic bones before entering the leg. In one patient an anomalous iliac artery was inadvertently divided during reconstruction of a cloaca; when recognized, the vessel was rejoined. Recognition of an association between urogenital or cloacal malformations and a major vascular anomaly is of great importance to reconstructive surgeons who, if not forewarned, could unintentionally compromise the arterial supply to one or both lower limbs.

Abnormalities, Multiple↗
PubMed1993

Fetal neuroblastoma: prenatal diagnosis and natural history.

Obstetrical sonography has helped diagnose and define the features of some congenital malformations and tumors. We present five fetal neuroblastomas detected by routine prenatal sonography. All were adrenal tumors diagnosed between 26 and 39 weeks gestation. All 5 tumors were completely resected postnatally and the patients have remained disease free from 2 months to 10 years after resection without adjuvant therapy. A literature review collated 16 other cases of fetal neuroblastoma detected by sonography between 29 and 38 weeks gestation. These cases included 1 cervical, 1 thoracic, and 14 adrenal tumors. Thirteen neonates had Evans stage I or II tumors, and three had more advanced disease. Eleven mothers did not have hypertension or preeclampsia during the pregnancy, and the neonates all had stage I or II disease. Four mothers had hypertension or preeclampsia. Three of these neonates had stage IV or IVS disease with liver metastases, and all three had fetal hydrops. Review of the congenital neuroblastoma literature documented 71 cases diagnosed soon after birth, and several of these cases had unusual features that could have been detected by prenatal ultrasound. Four of the tumors were so large that dystocia resulted and fetal dismemberment was required for delivery. Eight of the tumors metastasized to the placenta, and 1 metastasized to the umbilical cord with subsequent fetal death. We conclude that fetal neuroblastoma can be diagnosed by prenatal sonography. Accurate staging is difficult by sonography, but in mothers with no preeclampsia symptoms the chance of widely disseminated disease is small.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Posterior mediastinal masses.

Solid mediastinal masses in infancy and childhood occur most frequently in the posterior mediastinum. From 1972 to 1989, 63 patients presented with a posterior mediastinal mass. The median age at diagnosis was 6 years (range, 1 day to 26 years). Thirty patients were female. Forty-five percent of the patients presented with respiratory symptoms or chest pain; 13% had neurologic symptoms, one half of which were related to spinal cord compression; and 5% had a palpable mass. In 32% of patients the mass was an incidental finding. The tumors were of neurogenic origin in 89% of patients, of which neuroblastoma was the most common. Of all patients with posterior mediastinal masses, 60% had malignant tumors. Median follow-up for 62 of 63 evaluable patients was 45 months (range, 1 to 289 months). One patient was lost to follow-up. Of the 62 patients followed, 84% are alive and free of disease. All but 4 of the 32 patients with neuroblastoma are alive and free of disease with a median follow-up of 73 months (range, 7 to 289 months). Patients with neuroblastoma who were diagnosed in the first year of life had a significantly better survival pattern than those presenting after the first year. There were seven deaths in the series: four from neuroblastoma, two from primitive neuroectodermal tumor, and one from malignant schwannoma. Preoperative diagnostic evaluation of a posterior mediastinal mass should include posteroanterior and lateral chest roentgenograms, and either CT or MRI of the chest and abdomen to assess the extent of the mass.(ABSTRACT TRUNCATED AT 250 WORDS)

Adolescent↗
PubMed1993

Cloacal exstrophy: experience with 20 cases.

Exstrophy of the cloaca, a rare anomaly, is among the most complex malformations of infancy. Features include omphalocele, imperforate anus, and exstrophy of two hemibladders, between which lies the everted cecum. A small colon ends blindly in the pelvis, and the terminal ileum often prolapses out of the exposed cecum. Formerly, most of these infants died. From 1974 to 1992, 20 of these patients were treated, the majority in the past decade. Six were managed primarily; 14 were referred after prior surgery elsewhere. Current treatment of a newborn with cloacal exstrophy includes closure of the omphalocele, separation of the gastro-intestinal tract from the hemibladders, and closure of the two hemibladders as a single viscus. The colon can be pulled through in some cases, either immediately or later (6 cases). The bladder must be augmented in all cases to provide adequate volume and compliance; stomach is ideal for this (10 cases). A bladder continence mechanism must be constructed by narrowing the outlet (9 cases), or inserting a reversed small bowel nipple (7 cases). Intermittent catheterization is needed for the patient to empty the reconstructed bladder. Although two thirds of these patients are genetic males, they should be raised as females because they lack adequate tissue to construct a phallus. Magnetic resonance imaging of the spine has shown tethering of the cord in all patients; most were released neurosurgically. Lessons provided by these cases demonstrate that a satisfactory surgical and social outcome can be achieved today in most children with cloacal exstrophy.

Abnormalities, Multiple↗
PubMed1993

The effect of gastric augmentation on bladder function.

Gastrocystoplasty was performed in 29 female and 14 male patients 1 to 23 years old (average age 11 years). Urodynamic studies were completed in 20 patients preoperatively and within 6 months postoperatively. When postoperative studies were compared with the preoperative evaluation, it was noted that overall capacity increased an average of 220% (range 20 to 750%). Compliance also improved dramatically postoperatively. Bladder pressures increased from a mean volume of 34% of capacity preoperatively to 73% of the post-augment capacity. The average maximum filling pressure decreased by 32%, from 57 to 39 cm. water. Uninhibited contractions resolved completely in 6 of 10 patients who had them preoperatively. No one had uninhibited contractions postoperatively. Cyclical contractions of the stomach segment not exceeding 50 cm. water developed in 6 patients. The gastric patch seems to be an adequate source for improving the dynamics of a poorly functioning detrusor muscle in most patients. When a diamond-shaped patch of stomach is harvested and added to the bladder a spherical configuration is created, enhancing the storage properties. This result is in sharp contrast to other bowel segments, which must be detubularized to produce a suitable reservoir. The long-term effects of gastric augmentation remain to be determined.

Adolescent↗
PubMed1993

Tethered spinal cord in patients with anorectal and urogenital malformations.

We reviewed 26 patients with anorectal or urogenital malformations managed by the neurosurgical service at The Children's Hospital for tethered spinal cord as diagnosed by spinal MRI. There were 13 patients with cloacal exstrophy, 5 with the Vater association, 3 with imperforate anus, and 5 with other complex anomalies. Tethering spinal cord lesions included myelocystocele, lipomyelomeningocele, and filum lipoma. We review the clinical course, management, correlation of MRI and intraoperative findings, and outcome of the patients, as well as the embryology which underlies the association of these malformations.

Adolescent↗
PubMed1992

Pneumatocele complicating hyperimmunoglobulin E syndrome (Job's Syndrome).

The case of a boy with hyperimmunoglobulin E syndrome or Job's syndrome is presented to demonstrate the occurrence of pneumatoceles in this syndrome as well as their unusual natural history and failure to spontaneously resolve. Surgical resection was required for two complications in this patient, persistent bronchopleural fistula and a pulmonary abscess that destroyed one lung and required pneumonectomy. Pathologic examination of the specimens demonstrated the wall of the cysts consisted of granulation tissue with chronic active inflammation surrounded by infarcted pulmonary parenchyma with coagulative necrosis. The mechanism responsible for increased immunoglobulin E production in this syndrome is unknown, as is the manner in which elevated immunoglobulin E levels impair normal immune function.

Aspergillosis↗
PubMed1992

Cloacal malformations: experience with 105 cases.

A personal experience with 105 cloacal malformations is described, showing the wide range of anatomy that can occur with the urogenital sinus, the vagina(s), and the rectum, as well as in the external features of the perineum. Follow-up is presented on 98 patients, excluding 4 who died preoperatively, 2 who are preoperative, and 1 lost to follow-up but never repaired. Fifty-seven patients had vesicoureteral reflux. Data are given on urinary control, bowel control, and sexual function for those who are now adults. Two have had babies. Operation often requires multiple positions, including the posterior sagittal approach, laparotomy, and lithotomy position. Isolated rectal pull-through should never be performed in these patients, because the urogenital aspects of the malformations are the most difficult to repair and are the most life-threatening to the patient. Six recent cases are presented to illustrate the breadth of the cloacal spectrum. In most of these patients a satisfactory functional result can be achieved for urinary and bowel control as well as sexual function.

Abnormalities, Multiple↗
PubMed1992

Ileal nipple for continence in cloacal exstrophy.

Since 1985, 7 children 2 to 17 years old with urinary incontinence from cloacal exstrophy underwent bladder augmentation and creation of a small bowel nipple at the bladder outlet. There were 3 genetic female and 4 genetic male patients being reared in the female role. In 3 early cases augmentation was done with small bowel, while gastric augmentation was used in 3 later cases and 1 had both types. Six patients became completely dry and they empty by intermittent self-catheterization. In the youngest patient the operation failed because the nipple became infarcted and disappeared. Reoperation will be performed after at least 1 year has passed. In cloacal exstrophy if there is insufficient local tissue to create a bladder neck and urethra, a reversed, catheterizable bowel nipple may be an option to consider.

Abnormalities, Multiple↗
PubMed1992

Perforation of the augmented bladder.

In 12 of 264 children treated with enterocystoplasty 15 spontaneous perforations occurred. Of the 12 children 9 had myelodysplasia. All segments of the gastrointestinal tract were used for the augmentation and most were detubularized. Surgery to increase bladder outlet resistance was done in 8 cases. At the time of each perforation 9 children had sterile cultures, however, 3 died of overwhelming sepsis. Presenting signs included abdominal pain in 8 cases, septic shock in 4 cases and shoulder pain in 4 older myelodysplastic children with diaphragmatic irritation from escaping urine. Cystography demonstrated a leak in 10 of 11 cases. Urodynamic studies revealed good compliance with low maximum filling pressure in 8 of 10 children. Hyperreflexia was noted in only 5 cases and outlet resistance greater than 85 cm. water was demonstrated in 5. Histological analysis showed changes in the bowel wall consistent with ischemia but suture granulomas were present in areas adjacent to the perforation site or thinned areas in biopsy or autopsy specimens. In addition to the theory that overdistention may cause enterocystoplasty perforation, current detubularization techniques may produce areas of relative ischemia, which become accentuated when the augmented bladder is distended beyond a reasonable volume.

Adolescent↗
PubMed1992

Chordee without hypospadias: experience with 33 cases.

We treated 33 patients 6 months to 19 years old with penile chordee without hypospadias from 1966 to 1990. In all cases the penile shaft was degloved, the urethra was widely mobilized and chordee was resected. The shaft skin then was closed, usually using Byars' flaps to shift some preputial skin ventrally. This method sufficed to straighten the penis in 10 patients. In 3 patients urethral mobilization plus placement of a dermal graft to the shaft accomplished straightening of the penis. A total of 20 patients also required lengthening of the penile urethra. Lengthening was done with a graft taken from the prepuce in 14 patients, bladder in 2 and arm in 4. Four of those patients also required a dermal graft to the shaft. There were 8 complications in 7 patients, including anastomotic stenosis, ballooning of a graft, balanitis xerotica obliterans in a graft, urethral fistula and persistent chordee. Reoperation corrected each complication. All end results were satisfactory.

Adolescent↗