Polyorchidism with presumed contralateral intrauterine testicular torsion.

INTRODUCTION: Polyorchidism was first described by Blasius in 1670(1) during a routine autopsy. We report a child with unilateral polyorchidism and a contralateral absent testis, a combination not reported previously. PRESENTATION OF CASE: A 2-year-old boy was referred to the outpatient clinic with an impalpable left testis. At laparoscopy, the left vas deferens and testicular vessels ended blindly proximal to a closed internal ring. No gonadal tissue was identified. On the right side, a single vas deferens and testicular vessels were seen entering the internal ring as normal. The right side of the scrotum was explored and two testes were identified within a single tunica vaginalis. DISCUSSION: Polyorchidism is rare with a literature search identifying approximately 230 reported cases. Whilst prenatal testicular torsion is increasing being recognized and treated as a surgical emergency,(9) prenatal testicular torsion in association with polyorchidism has not been previously reported. CONCLUSION: We describe a unique case of a 2-year-old boy with right-sided polyorchidism and an absent left testis associated with a blind ending vas deferens and testicular vessels, presumed secondary to intrauterine testicular torsion.

Establishing benchmarks for the outcome of herniotomy in children.

BACKGROUND: Although complication rates following herniotomy in children are low, reported rates vary. The aim of this study was to propose desirable outcome measures that can be used by surgeons to assess the quality of their service. METHODS: A review of prospectively collected data on all hernias operated on by the Christchurch-based paediatric surgical service was undertaken. Complication rates were determined and compared with those reported in the literature. Rates for children aged 1 year or above were compared with those in children aged less than 1 year. RESULTS: There were 3128 herniotomies performed, with an overall recurrence rate of 0.6 per cent. Wound infections occurred in 0.9 per cent, but were confined to infants aged less than 12 months. The rate of acquired cryptorchidism was 0.4 per cent and testicular atrophy 0.8 per cent in boys. Injury to the vas deferens was recognized in 0.1 per cent. Children under 1 year of age were more likely to develop recurrence than older children (0.9 versus 0.2 per cent respectively; odds ratio 4.56, P = 0.013). CONCLUSION: Complication rates were relatively low compared with those reported previously, but were considered achievable in most paediatric surgical units. Specific attention should be paid to the youngest infants, as this group has the highest complication rate. Minimum standards can be proposed based on these and other published outcomes.

The degree to which the size and side of an inguinal hernia is predictive of a hernia on the other side.

BACKGROUND: The indication for contralateral exploration in patients with a unilateral inguinal hernia and the implications of a contralateral patent processus vaginalis have been the subject of much debate during recent decades. The aim of this study was to investigate whether the size and side of the hernial sac on the side of presentation are predictive for the operative appearance of the processus vaginalis/hernial sac on the contralateral side. METHODS: All primary herniotomies performed by the Christchurch-based paediatric surgical service were reviewed. Patients who developed a contralateral hernia after unilateral repair were identified. In a random subset of patients who had bilateral exploration for unilateral hernias, the size of the clinical hernial sac was plotted against the size of the contralateral patent processus vaginalis/hernial sac (when present) as assessed at the time of open surgery. RESULTS: Overall, 2,124 unique patients underwent herniotomies in the period reviewed, and 44 (3.8%) metachronous contralateral hernias were identified. Girls were not at greater risk than boys of developing a metachronous hernia. There was a positive correlation between the size of the clinical hernial sac and the size of the contralateral processus vaginalis/hernial sac. There was no significant relationship between the original presenting side and the development of a metachronous contralateral hernia. CONCLUSION: It is not possible to predict reliably which patients who present with a unilateral hernia would benefit from bilateral exploration, although the size of the hernia has a positive correlation with the size of the contralateral processus vaginalis/hernial sac. Those with "massive" hernias on the presenting side were more likely to have "large" contralateral hernial sacs, but how many of these would subsequently become symptomatic remains uncertain.

The development of the proximal oesophageal pouch in the adriamycin rat model of oesophageal atresia with tracheo-oesophageal fistula.

This study examined the morphological development of the proximal oesophagus in the Adriamycin-induced rat model of oesophageal atresia. The proximal oesophageal segment in oesophageal atresia with tracheo-oesophageal fistula (OA\TOF) has been assumed to be of similar embryological origin to the distal oesophagus. However, recent research using the Adriamycin model of OA\TOF has indicated that these structures may have a different origin. Time-mated Sprague-Dawley rats were administered either Adriamycin intraperitoneally or saline of an equivalent volume between days 6-9 of gestation. The rats were sacrificed between days 11-19 of gestation, their embryos removed and histologically sectioned. These sections were analysed to observe the morphological changes occurring in the proximal foregut. The proximal oesophageal pouch first appeared on day 15.25 as a dorsal outpouching of the proximal foregut immediately cranial to an area of apoptosis in the dorsal epithelium of the distal pharynx. It elongated through a process of cellular proliferation until it was clearly formed on day 16. Relatively little growth occurred from days 17-19. In the rat developing oesophageal atresia, the proximal oesophageal pouch has an origin different to that of the distal oesophagus. This study may explain the difference in immunohistological properties and intrinsic nervous supply between the proximal and distal oesophageal segments in oesophageal atresia.

Fatal late onset necrotising enterocolitis in a term infant: Atypical Kawasaki disease or polyarteritis nodosa of infancy?

A 3-month-old infant died following a 3-week illness that commenced with diarrhoea and vomiting, and produced progressive infarction of his small bowel. Post-mortem identified multiorgan arteritis of uncertain aetiology, giving rise to coronary artery occlusion and myocardial infarction as well as necrosis of the entire small bowel and other organs. Kawasaki disease and polyarteritis nodosa of infancy are proposed as possible aetiologies in this case.

Familial parathyroid carcinoma in a child.

Familial hyperparathyroidism and parathyroid carcinoma are rare diseases. A case of parathyroid carcinoma in an 8-year-old girl whose mother had previously undergone parathyroidectomy for primary hyperparathyroidism is reported. Parathyroid carcinoma in a preadolescent child has not been described previously, and may have a familial basis.

Hedgehog in the human: a possible explanation for the VATER association.

Foregut malformations are relatively common anomalies, occurring in 1 in 2000-5000 live births. The adriamycin-induced rat model of the VATER association has provided a means of studying the morphogenesis of a variety of major congenital structural abnormalities similar to those seen in humans with VATER association. The secreted glycoprotein, Sonic hedgehog (Shh), may act as an endodermal signal that controls gut and lung patterning. Mice with targeted deletion of Shh have foregut defects that are consistent with those produced by administration of adriamycin. It is possible that mutations induced by adriamycin may result from the breakdown of the Shh signalling pathway.

Abnormal branching and regression of the notochord and its relationship to foregut abnormalities.

An abnormally positioned notochord has been reported in embryos that develop foregut abnormalities, vertebral defects and other abnormalities of the VATER association. This study examines the patterns of regression of the abnormal notochord in the rat model of the VATER association and investigates the relationship between developmental abnormalities of the notochord and those of the vertebra and foregut. Timed-pregnant Sprague-Dawley rats were given daily intraperitoneal injections of 1.75 mg/kg adriamycin on gestational days 6 - 9 inclusive. Rats were sacrificed between days 14 and 20 and their embryos harvested, histologically sectioned and stained and examined serially. The location and appearance of the degenerating notochord and its relationship to regional structural defects were analysed. All 26 embryos exposed to adriamycin developed foregut abnormalities and had an abnormal notochord. The notochord disappeared by a process of apoptotic degeneration that lagged behind that of the normal embryo: the notochord persisted in the abnormal embryo beyond day 17, whereas in the normal rat it had already disappeared. Similarly, formation of the nucleus pulposus was delayed. Vertebral abnormalities occurred when the notochord was ventrally-positioned. The notochord disappears during day 16 in the normal embryo whereas abnormal branches of the notochord persist until day 19 in the adriamycin-treated embryo. Degeneration of the notochord is dominated by apoptosis. An excessively ventrally-placed notochord is closely associated with abnormalities of the vertebral column, especially hemivertebrae.

Evidence of a common pathogenesis for foregut duplications and esophageal atresia with tracheo-esophageal fistula.

The pathogenesis of the alimentary tract duplications, including foregut duplications (FgD) remains speculative. The accidental finding of FgD in fetal rats with esophageal atresia and tracheoesophageal fistula (EA-TEF) induced by Adriamycin provided an animal model to investigate a possible relationship between these two entities. Timed-pregnant rats were intraperitoneally injected with Adriamycin (1.75 mg/kg) on gestational Days 6 to 9. Their embryos were harvested by Caesarean section from gestational Days 14 to 21. Forty-six of embryos were processed and serially sectioned in the transverse or sagittal planes. EA-TEF occurred in 43/46 (93%) embryos of which 11 (24%) were found to have an associated FgD located at the level where the esophagus was absent. Six FgDs communicated with the foregut or the trachea. Five noncommunicating FgDs were located between the foregut and the vertebral column. In the control embryo, the notochord was located in the centre of the vertebral column from Day 11 of the gestation. In Day 14, 15 and 16, however, embryos exposed to Adriamycin, an abnormal notochord or branch frequently was located within the mesenchyme of the maldeveloped foregut or attached to the duplication cyst. In some, it appeared that the notochord was drawing the cyst-like structure away from the foregut. The present study confirms that duplications adjacent to the esophagus arise from the foregut and that failure of the foregut to detach from the notochord at the normal time may contribute to the development of foregut duplications.

Morbidity associated with gastrostomy placement in children demands an ongoing integrated approach to care.

AIMS: To evaluate the short and long term morbidity of gastrostomy insertion, and to identify ongoing management requirements. METHODS: A retrospective review was undertaken of the hospital casenotes of children aged up to fifteen years who had a gastrostomy placed in Christchurch over a six year period to March 1998. RESULTS: 42 children had a gastrostomy fashioned, 35 in the last three years of the period reviewed. The most common underlying diagnosis was neurological disease (48%), and the most common indication for tube placement was failure to feed orally. Complications were frequent but minor. Morbidity was related to local erythema and infection around the stoma (85 episodes in 23 children), persistent and major gastric fluid leakage (three episodes), and mechanical failure of the tube (21 episodes). Gastro-oesophageal reflux was seen in fourteen children, nine of whom had primary neurological disease. Complications were seen more after open gastrostomy than after percutaneous endoscopic placement (6.6:4.7). There was no mortality related directly to the gastrostomy tube or tube placement. CONCLUSIONS: An increase in the frequency of gastrostomy placements has been seen over this period. As the number of children with a gastrostomy increases, so too have the demands on medical and nursing staff to care for and manage the devices. The frequency of minor ongoing problems necessitates ongoing support of the child and care of the gastrostomy. A close working relationship between outreach nursing staff, stoma therapists and medical staff is required if morbidity is to be minimised. Education, audit and review remain important additional aspects of care.

Demonstration of abnormal notochord development by three-dimensional reconstructive imaging in the rat model of esophageal atresia.

The notochord (Nt) is believed to have a role in the development of axial organs. This study was undertaken to reconstruct in three dimensions (3D) the relationship of the Nt to abnormal development of the foregut (Fg) in the adriamycin-induced rat model of esophageal atresia (EA). Pregnant Sprague-Dawley rats were given 1.75 mg/kg adriamycin intraperitoneally on gestational days 6 9 inclusive; control rats received i.p. saline of equal volume, or no injection. Rats were killed between days 11 and 14 and their embryos harvested, histologically sectioned serially, and stained with hematoxylin and eosin. Digitized photographs were taken of serial transverse sections; these photos were traced and used as the basis for 3D reconstruction. From day 11 the normal Nt is no longer in contact with the respiratory or Fg mesenchyme. In adriamycin-treated embryos the Nt branches abnormally as it enters the Fg mesenchyme. Adherence of the Nt to the mesenchyme of the Fg exerts mechanical traction pulling the upper Fg dorsally. The severity of the Fg abnormalities correlates with the length of the ventral extension of the Nt within the Fg mesenchyme: the embryo develops atresia of the esophagus or trachea when the Nt is grossly abnormal. The Nt undergoes reactive thickening in the absence of Fg structures ventral to it. Thus, structural lesions of the Fg (e.g., atresias) are associated with abnormalities of the Nt. The relationship of the Nt to the Fg mesenchyme determines the severity of the abnormality induced by adriamycin: extensive adherence produces tracheal agenesis and EA.

Abnormalities of the tracheal cartilage in the rat fetus with tracheo-oesophageal fistula or tracheal agenesis.

Many infants with oesophageal atresia and tracheo-oesophageal fistula (OA/TOF) have associated tracheomalacia (TM), which is one of the reasons for respiratory complications after surgical correction of the atresia. OA/TOF was induced in the offspring of pregnant rats by intraperitoneal injection of adriamycin. Fetuses were harvested by caesarean section. The trachea, oesophagus, lungs, and stomach were removed en bloc and stained for cartilage using Alcian blue. The tracheas were examined, photographed, and relevant parameters pertaining to the tracheal cartilage were measured. Exposure to adriamycin resulted in a range of anatomical defects including OA/TOF (47%) and tracheal agenesis (TA) (41%). Adriamycin-treated fetuses were smaller (P < 0.01), yet had longer tracheas (P < 0.001) than control fetuses. The OA/TOF fetuses had more tracheal cartilage rings than controls (P < 0.01), whereas TA fetuses had fewer (P < 0.001). Both OA/TOF and TA fetuses had more malformed tracheal cartilage rings than controls (P < 0.001 and P < 0.05, respectively). Cartilage in the proximal part of the trachea was most frequently and severely affected (P < 0.05). These observations clarify the structural abnormalities of tracheal cartilage that occur in rat fetuses with OA/TOF or TA induced by adriamycin, and may explain the functional disturbances of TM seen in OA/TOF.

Does anorectal manometry predict clinical outcome after laparoscopic ACE procedures in children with spina bifida?

PURPOSE: The laparoscopic antegrade continence enema (LACE) procedure is used for the treatment of faecal incontinence in children with spina bifida. The purpose of this study was to relate the outcome of the LACE procedure to anorectal function, as determined by anorectal manometry. METHOD: Eleven children with spina bifida who had the LACE procedure underwent anorectal manometry (ARM) to document their anorectal function, and its relationship to the level of continence obtained following surgery. RESULTS: There was a consistently high level of functional continence achieved following surgery despite wide variability in the parameters of anorectal manometry. There was no demonstrable correlation between the outcome of the LACE procedure and anorectal function as assessed by manometry. CONCLUSION: Anorectal function as determined by manometry failed to predict outcome after the laparoscopic ACE procedure in children with spina bifida.

The contribution of the adriamycin-induced rat model of the VATER association to our understanding of congenital abnormalities and their embryogenesis.

The adriamycin-induced rat model of the VATER association has provided a means of studying the morphogenesis of a variety of major congenital structural abnormalities similar to those seen in humans with the VATER association. Most interest has been centered on the foregut, where the model has clarified some aspects of the development of esophageal atresia (EA), tracheal agenesis, and other communicating bronchopulmonary foregut malformations. It has demonstrated aberrations in the nerve supply to the esophagus in EA and allowed the study of tracheomalacia. A relationship between an abnormal notochord, foregut abnormalities, and vertebral defects has been shown, and the model has reignited interest in the role of the notochord as a regional organizer of axial development. The normal temporospatial characteristics of apoptosis during fore- and hindgut development is disturbed in this model, resulting in abnormal morphology. The indications are that this model will continue to clarify the processes that lead to many of the structural congenital abnormalities that are seen in infants born with the VATER association.

The effect of the availability of laparoscopic techniques on the treatment of appendicitis in children.

The use of laparoscopic surgery for acute appendicitis (AA) in children has increased over recent years. The aim of this study was to determine what effect the availability of laparoscopic appendicectomy (LA) has had on children admitted with suspected AA. A retrospective review of children admitted between January 1994 and June 1999 inclusive who underwent appendicectomy for suspected AA was conducted. Data recorded included standard demographic information, surgical approach, histopathology of the appendix, complications, and post-operative length of stay. After LA, children had a shorter post-operative stay, although the technique was used less frequently in advanced disease. The rate of normal histology was higher for LA. Laparoscopic surgery was performed in females in 69% of whom 48% had a normal appendix removed. The duration of surgery was longer for LA (59 vs 40 min). The rate of LA increased to 77% in the first 6 months of 1999. There was a decrease in the proportion of laparoscopic procedures converted to open appendicectomy from 50 to 6% during the period reviewed. The ready availability of laparoscopy and increased confidence in its use has resulted in more children, especially females, with suspected AA undergoing laparoscopy. There was a corresponding higher rate of normal appendix removal in this group, but the overall rate of normal histology has not changed, suggesting that the laparoscopic approach is more likely to be employed where the clinical diagnosis is less certain, particularly in older girls.

Does the urorectal septum fuse with the cloacal membrane?

PURPOSE: Traditional theories of cloacal embryogenesis assume that the urorectal septum fuses with the cloacal membrane before the anal membrane disintegrates. However, recent observations in humans and other species raise doubt about this assumption. We determined whether urorectal septum fusion occurs in rats. MATERIALS AND METHODS: Rat embryos were harvested at specific times between days 11 and 16 of gestation. We evaluated the morphology, growth and relationship of the urorectal septum to the cloacal membrane on serial histological sections. RESULTS: The urorectal septum consistently fused with the cloacal membrane on day 15 of gestation before the cloacal membrane began to disintegrate. CONCLUSIONS: In rats the urorectal septum fuses with the cloacal membrane, after which the urogenital membrane and anal membrane disintegrate by a process of apoptosis.

Apoptosis during regression of the tailgut and septation of the cloaca.

PURPOSE: Apoptosis is involved in the embryonic morphogenesis of many organs. The current study was undertaken to ascertain the role of apoptosis during cloacal development in the rat. METHODS: One hundred five rat embryos, ranging from gestational days 11 to 16, were sectioned serially in the transverse or sagittal planes and stained with H&E. The cloaca, urorectal septum, rectum, urogenital sinus, Wolffian ducts, and tailgut (TG) were examined consecutively in temporospatial sequence. RESULTS: The tailgut immediately distal to the hindgut starts to regress by apoptosis on day 12 of gestation in a craniocaudal direction and has regressed completely by day 13.5. A large number of apoptotic cells and debris can be identified in the urorectal septum during cloacal septation. Vacuoles are formed by coalescence of apoptotic cells at the tip of urogenital sinus from day 15 to 16, and, at the same time, sporadic apoptotic bodies in the anal membrane contribute to its thinning. CONCLUSION: Results of the current study confirm that apoptosis occurs in a specific temporo-spatial sequence in the hindgut and cloaca and appears to be an important mechanism in TG regression, uro-rectal separation, urethral opening, and rupture of the anal membrane.

Temporospatial aberrations of apoptosis in the rat embryo developing esophageal atresia.

BACKGROUND/PURPOSE: Recent work has shown that apoptosis is a key component of the normal development of the foregut. This study was designed to compare the patterns of apoptosis in the normal foregut with those in the fetus developing esophageal atresia and tracheoesophageal fistula (EA-TEF) using 3-dimensional reconstructive techniques. METHODS: Timed pregnant rats that received no treatment (control group) or received Adriamycin intraperitoneally (experimental group) had their embryos removed between days 11 and 14 of gestation. The embryos were sectioned serially and stained with H&E. Three-dimensional reconstructions were made of the foregut and areas of apoptosis were marked on them to facilitate analysis of apoptotic patterns. RESULTS: Apoptosis was evident in control embryos in the region in which tracheoesophageal separation occurs from days 12 and 12.5. Experimental embryos showed no apoptosis until day 13 when apoptosis was observed immediately posterior to the foregut within the esophageal mesenchyme and in the laryngeal mesenchyme ventral to the foregut. CONCLUSIONS: The pattern, timing and location of apoptosis in rats developing EA-TEF is abnormal. Our work indicates that it is actually a complete lack of apoptosis at the crucial stage of development that leads to this abnormality rather than an alteration in the patterning of apoptosis at this crucial time. The observation of apoptosis only within the mesenchyme raises the possibility that apoptosis in the foregut developing EA-TEF may be a reaction to that abnormal development rather than its cause.