Most patients with constipation are treated with a combination good diet, attention to toileting and the addition of medication in some. However, like many diseases, there is a range of the causes and the lengths to which one has to go to get a cure. Surgery has a limited but important role in constipation in the community. The following paper addresses the role of surgery.
Megarectum and Constipation
Professor Paddy Dewan
1. Definition of encopresis, soiling and constipation. 3
2. Definition of megarectum.. 4
3. Differential diagnoses. 5
4. Preoperative investigations. 6
Abdominal Radiograph. 6
Barium Enema. 6
Magnetic Resonance Imaging (MRI). 6
Rectal Biopsy. 7
Colonic transit time. 7
5. Type of surgery for constipation. 8
i. Malone stoma. 8
iii. Imbrication. 8
iv. Duhamel 8
v. Colo-anal anastomosis. 8
vi. Transanal Swenson’s. 8
vii. Transabdominal Swenson’s. 9
viii. Rectosigmoid resection. 9
ix. Vertical reduction rectoplasty. 9
x. Colostomy. 9
6. Timing of surgery for constipation. 10
7. Outcome for megarectum surgery. 12
1. Definition of encopresis, soiling and constipation
The term encopresis has been used to describe the “diagnosis” of the patients but, like the word “constipation”, the term does not define a disease, as it is merely a descriptive term. Van der Plas et al., in their study to improve the understanding of what constitutes a megarectum, define encopresis as the loss of a normal amount of stool in the underwear after the age of 4 years, without any underlying organic disorder (van der Plas 2000), as did Benninga et al.(Benninga 1994). Chiarioni et al., in assessing a megarectum in children manometrically, restated the definition used by van der Plas et al., highlighting that encopresis is in the absence of organic disorder (Chiarioni 2005). Alberto Pena suggested that the encopretic does not even have spontaneous bowel motions (Pena 2002), but has soiling. Megarectum is also a descriptive term, which when used implies a rectum that is so abnormally large that it will not return to normal with medical management, others use the term to mean “big”, which indicates one of the reasons there is such confusion about good care of individual patients with severe constipation.
Benninga et al. note the important difference between encopresis and soiling as the amount of faeces lost, soiling being the lesser amount of the two. They note that the terms are inappropriately used indistinguishably in published work. Faecal soiling represents the staining of underwear and is often associated with faecal impaction, is the common surgical use of the terms. They were adamant that, by definition, there is no faecal mass palpable in the encopretic, a group they appropriately considered would respond to behaviour treatment (Benninga 1994). They go on to assert that encopresis can occur without any constipation, which is commonly understood.
Nixon, a paediatric surgeon of repute from Great Ormond Street Children’s Hospital stated, “Constipation means incomplete emptying; encopresis is not overflow and there is no marked constipation or megarectum” (Nixon 1967). Constipation is defined in many ways, but most accept that constipation is a term that does not equate to a diagnosis, but does imply infrequent stools, incomplete emptying, firm stools, straining at stool, pain on defaecation and abdominal pain or a combination of some or all of these. Certainly, there is no literature support for a boy presenting with a rectum loaded with faeces, occupying most of the width of the pelvis, as never having had constipation.
According to Coccorullo et al., treatment of constipation is still largely based on clinical experience rather than on evidence-based studies (Coccorullo 2009).
Many authors provide comment about the common experience with children, who pass faeces into their underwear, with or without associated constipation, stating that they “just don’t feel it”. It is also well known that children will tolerate the odour of soiling without recourse to self cleaning. Oppe, as early as 1967, noted that “another aspect of denial is the claim made by the child that he is unaware of soiling or the presence of the faeces in the rectum" (Oppe 1967), which anyone involved in the management of faecal and urinary incontinence children should be very conversant with – i.e., children with any form of incontinence will say “I didn’t feel it”, despite normal sensation.
2. Definition of megarectum
Before discussing megarectum, let me explore the presence of dilated segments of other parts of the internal anatomy. The renal pelvis is considered to require resection of the baggy pelvis when it has been dilated by obstruction, rather than just the obstruction being surgically resolved. Likewise, the oesophagus and duodenum are plicated as part of the management of congenital anomalies, to resolve the problem with the persistent dilated segment. In 1990, Dewan and Guiney described a case of a girl with segmental dilatation of the duodenum, who presented with failure of pubertal development, which was resolved by resection of the dilated component. Dr Adrian Bianchi, a Paediatric Surgeon from Manchester, has provided a technique for dealing with ectatic segments of small bowel (Davenport 1990), and Swenson and Rathauser in 1959, presented the first three cases of segmental dilatation of the colon and rectum (Swenson 1959) that were treated with resection; further evidence of the role of ectatic intestinal segment surgery benefit, since when Etzioni presented a case of proximal colonic segment dilatation in a three year old, in 1980 (Etzioni 1980). In 2004, Al-Salem reviewed 12 cases of segmental colon and/or rectal dilatation, noting that 10 of the 12 cases included were operated on at less than 10 years of age (Al-Salem 1990).
What about megarectum? Unfortunately, megarectum is a term that is used across a wide spectrum of abnormality, as mentioned, and akin to broadbrush terms like constipation, asthma or a long list of such minimally informative terms. Megarectum alone does not define the size of the rectum, the nature of the aetiology, the prognosis, nor the therapy required for the individual patient. Several authors who have attempted to define “megarectum” on barium study, but this only deal with one dimension of the meaning of the term. The first to suggest a definition was Preston, who concluded that a rectal diameter of greater than 6.5 cm in the lateral view was the lower limit above which the rectum was abnormal in adults (Preston 1985; Gattuso 1997). This lacks the clinical definition of the term, noting that treatment is rarely defined by the radiology alone. In Preston et al.’s study they investigated 50 controls, 18 slow transit constipation women and 20 idiopathic colon patients; those with slow transit constipation had a normal rectum, neither the controls, nor the slow transit constipation group had a rectal diameter above 6.5cm. The rectal width was measured from true lateral pelvic radiographs along a line drawn perpendicular to the body of S2. The bowel width was measured perpendicular to the lumen at the pelvic brim. (Preston 1985). In particular, the rectal diameter should be measured on a line extending perpendicular to the second sacral segment, which was not the standard applied by Dr Davies in his presentation in the Medical Board, which was also at variance with Gladman et al. who highlight that the rectal diameter should be the measured at 90 degrees across the widest transverse diameter at the pelvic brim (Gladman 2006). Several authors have since concurred on the definition of megarectum (Williams 2000; Chiarioni 2005), while others suggest 6.1 cm as the upper limit of normal (Elawad 2001; Griffin 2008) or add consideration of the extension of the megarectum from the pelvis (Godbole 2001): the younger the child the lower the expected upper limit of the rectal size. Appropriately, the absolute size of the rectum is not considered a good guide in paediatric patients, thus a recto-pelvic ratio of 0.61 is considered abnormal (van der Plas 2000). Some authors maintain that rectal function seems severely deranged due to the atonic state of the rectum that sometimes adapted to accommodate over 800ml (Chiarioni 2005). There isn’t a good publication of the clinical definition for a rectum that will not involute over time, but clinical experience can assist with the inference that medical management alone will not suffice.
There are several studies that have diagnosed and treated children with anorectal anomalies and a megarectum (Amano 1985; Hata 1986; Lee 2002; Kubota 2008; Levitt 2009) and many studies that have provided surgical care for adults with a diagnosis of chronic constipation and a megarectum (Stabile 1991; Stabile 1992; Gladman 2006), in doing so there has not only been evidence accumulated as to a radiological and manometric diagnosis, but also a histological confirmation. Brent and Stephens provide histologic evidence for primary rectal ectasia (Brent 1976) and, in 1997, Gattuso et al. reported on the histology of idiopathic megarectum in cases of rectal only or combined colonic dilatation, showing significant thickening of the megarectum muscularis mucosae (median 78microm vs. 73microm, p < 0.005), circular muscle (1000microm v 633microm, p < 0.005), and longitudinal muscle (1083 v 303 microm p < 0.005), despite rectal dilatation. The density of neural tissue in the longitudinal muscle seemed to be reduced in patients with idiopathic megarectum compared to normal controls (Gattuso 1997). Despite thickening, the megarectum sufferer tends to have an atonic rectum, able to tolerate large volumes that are too large to be defaecated (Chiarioni 2005). In patients with faecal impaction the rectal wall develops myohypertrophy in an attempt to overcome chronic faecal loading (van der Plas 2000), but whether is was congenitally abnormal is usually uncertain in those presenting later in life.
In fact, one of the points of confusion, about the appropriate management of constipation, soiling and a megarectum, is when can the diagnosis of a congenital anomaly be made? The point that a congenital anomaly can present after many years is made by observations related to a congenital defect in the diaphragm. The defect can be associated with prenatal death or, in the other extreme, can present well beyond the second decade with little appendicitis in the chest. (Weber 1991).
3. Differential diagnoses
This paper is not meant to be exhaustive of the topic of the differential diagnosis of idiopathic megarectum, but will make the point of the importance of ultra-short segment Hirschsprung’s disease as a differential diagnosis and highlight why a diagnosis of Hirschsprung’s could be possible at the end of an operation, but not at the start. It also intends to indicate the inappropriateness of protracted medical management in the presence of likely failure of the non-surgical treatment.
Megarectum is commonly treated as functional constipation or confused with Hirschsprung’s (Al-Salem 2008). Far too much has been made of this differential diagnosis, which can be ascertained by rectal biopsy and anorectal pressure studies, but the studies are only indicated when sufficiently likely to warrant the investigations (Moore 1996; Pearl 1998). A sufficiently certain diagnosis of megarectum can reasonably be treated with a pull-through operation, recognising that some even doubt the assertion that ultra-short segment Hirschsprung’s is a valid pathological entity. Levitt and Pena have changed from doing a sigmoid resection for constipation with megarectum to the per-anal Swenson’s procedure, recognising that, in doing so, a separate rectal biopsy procedure is unnecessary. Al-Salem, in 1990, described a 3 year old with segmental dilatation, and states that the barium enema examination shows distension of the rectal ampulla and colon in idiopathic megacolon, but similar radiographic picture may exist as in short-segment Hirschsprung’s (Al-Salem 1990). If the surgery addresses both then the point is moot.
It appears that in patients with idiopathic megarectum, impaired motility is a result of chronic retention and overstretching of the bowel, whereas the opposite is true in patients with primary rectal ectasia (Lee 2002). That is, there is a different disease in each patient and there is a spectrum of each disease, which concurs with Pena’s view who maintains “the concept of a spectrum of disease cannot be over emphasised .... standard protocols render good results in a percentage of cases but always leave a group of patients who do not respond (Pena 2002).
Fuacheron, in investigating 154 patients, reported on 12 cases of megarectum, which they considered as one form of akinesia (Faucheron 2000), which in effect fits with the functional disruption suggested by others, and the range of pathology proffered by Pena (Pena 2002).
4. Preoperative investigations
The abdominal radiograph can be used as an air contrast study, and to view the bony anatomy for spinal fusion defects. The images, taken in the context of the clinical findings can assist, but with limitations how conclusive the information and cautioned by the radiation required.
The definition of a megarectum has been discussed. Of note, films consistent with those in “the case” are seen in articles as early as 1995 (Halligan 1995) and Al-Salem in 2008 (Al-Salem 2008). Halligan et al. refutes that bowel preparation be used in children as “bowel preparation is difficult and unnecessary for the diagnosis and may hinder the recognition of the transition zone (seen in Hirschsprung’s), which is the main differential diagnosis of megarectum (Halligan 1995). The appearance of the barium study of a megarectum is discussed above.
An MRI is a useful investigation for the lower spine in a patient who presents with faecal incontinence, but where the patient clearly has a rectal anatomical cause of their incontinence, and no other indications of neuropathy, most would suggest an MRI of the rectal anatomy is not warranted. Aslam et al. do not support the use of an MRI, even in those with a major anorectal anomaly (Aslam 1998) and, in 2002, publishing from California, Lee et al. (Lee 2002) operated on four children, ages 9 to 15 years, with a history of unremitting constipation and faecal incontinence who were referred for consideration of surgery. All patients had a normal lumbosacral MRI, yet still had surgery. All four patients had megarectum on a contrast enema and anorectal manometry was consistent with functional faecal retention, highlighting the appropriateness of not performing an MRI in such cases as the investigation does not assist with the diagnosis of a megarectum.
Many studies of megarectum management have performed rectal biopsy on their cases, and state that a rectal biopsy should be used prior to the resection surgery. This is not logical as inferred by Levitt and Pena in their 2009 paper, which would treat the majority of Hirschsprung’s disease children by their megarectum operation of choice, thus, as stated above, why submit the patient to a separate anaesthetic and a separate operation that will not change the management, especially when the barium study has the appearance stating that the biopsy will be normal (Levitt 2009). Al-Salem has a slightly more rational approach stating that rectal biopsy be used in doubtful cases (Al-Salem 2008). It is valid to know if there is any neuronal anomaly in the resected specimen, but this can be achieved on the resected rectosigmoid segment.
Many other studies mention that they perform the megarectum surgery when the biopsy has been shown to be normal, including Gera et al. (Gera 2009) and Godbole et al., who operated on three of 22 patients after having conducted a normal MRI and a normal rectal biopsy (Levitt 2009). Godbole et al. measured the recto-sphincteric reflex in some, performed a rectal biopsy in others, and both in a few of their 40 megarectum cases on whom they operated on 11 (Godbole 2001). In other words, not all authors perform a rectal biopsy in all cases of a megarectum, and in all cases of a megarectum, biopsy is actually not needed prior to surgery if the operation of choice is a rectosigmoidectomy. Most who have worked in developing countries, without the luxury of any available histopathology would agree that even typical Hirschsprung’s can be treated with clear thinking, an initial diverting colostomy and then pull-through.
Pena maintains that manometry is unhelpful in evaluating the paediatric constipated patients (Pena 2002); and O'Suilleabhain et al. insist that anorectal physiology, colonic transit and evacuation studies did not aid selection of the surgical procedure performed in 15 patients (O'Suilleabhain 2001). Pena’s comment is in keeping with Gladman et al., who found that that colonic transit time normalised in some patients after surgical treatment for megarectum (Gladman 2005).
Radiologic studies of bowel motility involve the use of radio markers that are ingested by the patient and followed through the entire gastrointestinal tract, either markers that are seen on plain radiographs, or the more recently developed radiopharmaceuticals. The information that these study provide, Pena argues, is already known clinically (i.e., the colon moves slowly). Therefore Pena does not feel that it really contributes (Pena 2002). Colonic transit time is supported as a tool by some, but in these papers they often do not differentiate between the colonic anomaly and slow peristalsis of the whole gut (de Lorijn 2004).
Professor John Hutson’s Melbourne, Royal Children’s group (with possibly 252 cases by 2006 (Langer 2007)) assert the importance of slow transit times in the diagnosis of slow transit constipation, or neuronal intestinal dysplasia, although other authors dispute the existence of the diagnosis (Pena 2002), and other centres record virtually no experience of the diagnosis, including Herndon et al. who had only one case in 127 children (Herndon 2004), Croaker found none in their 140 cases in Canberra (Croaker 2007). Hutson’s group has large numbers of patients surgically managed with the formation of a Malone stoma, and other surgeries, for the treatment of neural intestinal dysplasia (King 2005; Southwell 2005). Pena states that "we are rather sceptical about the diagnosis of intestinal neuronal dysplasia, concluding that there is no basic agreement amongst pathologists how to establish this specific histological diagnosis. In addition, topographic studies that describe the extension of this histologic disorder in different patients are missing. For a surgeon to propose a rational treatment of this condition, the surgeon should know the extent of the affected bowel that would be resected and, in theory that will cure the patient. This has never been accomplished. In addition, the symptoms of patients with neuronal intestinal dysplasia vary from patient to patient. The patients’ management strategies vary from laxatives to enemas to different resections, and the follow-up of the patients has not been consistent. Pena goes on to highlight that, “to complicate the problem even more, some patients recover spontaneously” (Pena 2002). Hutson’s group now advocate externally applied electrical therapy, have performed laparoscopic biopsies in over 200 patients.
5. Type of surgery for constipation
A wide range of surgical therapies exist for assistance with the management of patients with constipation. Obviously, they have varying roles, but the presentation of the option of colostomy for idiopathic constipation, while to me is an extreme, inappropriate step, is none-the-less one of the options that a surgeon should consider in the armamentarium for the care of the constipated patient, particularly given the latter discussion on life threatening complications of constipation.
There is evidence that a failure to diagnose and treat sufficiently aggressively in children can result in significant life-threatening complications
The surgical options that have been recommended in adults, children or both include. Note (iv) to (viii) are variations of the same option:
i. Malone stoma
ii. Left sided antegrade continent enema
v. Colo-anal anastomosis
vi. Transanal Swenson’s
vii. Transabdominal Swenson’s
viii. Rectosigmoid resection
ix. Vertical reduction rectoplasty
To understand the appropriateness of the decision in the index case we need to work through the above options, particularly the invasiveness of the formation of a colostomy and the potential complications of the Malone antegrade enema option, which has been suggested by witnesses as an innocuous alternative.
Firstly, let me discuss the literature related to surgical alternatives for resection of bowel in constipated patients, which ranges from total colectomy to permanent colostomy. The surgery ranges from evacuation of the bowel (Elawad 2001), which does not have any significant surgical risk, then rectal biopsy, myomectomy (Hata 1986; Moore 1996), which are not without the risk of bleeding and infection - Pena considers the latter alternative less than satisfactory (Pena 2002). The formation of a catheterisable stoma to the right colon, known at the Malone stoma (Herndon 2004; King 2005; Poirier 2007; Griffin 2008), or to the left colon, know as the Lace (Griffin 2008).
The rectal segment has been reduced in size through either rolling it in on itself in a process known as imbrication (Dewan 2004), or reduced in size longitudinally by vertical reduction rectoplasty (Williams 2000; Gladman 2005). Then there are a large number of publications that advocate the use of resection and anastomosis, using procedures that perform a resection focused on removal of the sigmoid colon (Lee 2002; Gera 2009), both during an open operation and laparoscopically. Levitt and Pena, having considered that sigmoid resection was sufficient, published in 2009 that they prefer a transanal approach to perform the same procedure as for “the case” in 15 children with idiopathic megarectum, having used a sigmoid resection technique in 17 children previously (Levitt 2009). Amano treated 6 of 20 children with a megarectum with resection (Amano 1985) and advocated anterior resection for the treatment of mega rectosigmoid as a safe and effective procedure for the management of a megarectum. (Hallows 2002).
Others have used another operation initially devised for Hirschsprung’s surgery, the Duhamel procedure (Al-Salem 1990; Stabile 1991; Godbole 2001; Al-Salem 2008), with colectomy of various degrees being mentioned by others (Stabile 1992; Halligan 1995; Williams 2000; O'Suilleabhain 2001).
Colostomy and ileostomy have been used for the treatment of constipation, which seems extreme, but is obviously within the realm of reasonable care given that papers from noted institutions have been published in peer reviewed journals, including the paper by Woodward on 10 children (Elawad 2001; O'Suilleabhain 2001; Woodward 2003; Scarpa 2005). Perhaps these could be avoided by more proactive treatment in childhood.
It is important to further discuss the use of the Malone stoma, particularly to highlight that it is not the innocuous procedure suggested by some. Many papers have reported the use of surgery for constipation. A number of centres use the Malone stoma alone, but there is often not good definition of the differences between patients (Herndon 2004; King 2005; Poirier 2007; Griffin 2008). Lee et al. form a Malone stoma at the time of the resection of the megarectum, which is only used for a period of 12 months (Lee 2002). I would argue that it is seldom necessary after appropriate patient selection and extent of surgery. The original paper on the Malone stoma used the technique of attaching the appendix to the skin to facilitate regular washout in only 5 patients from October 1989, three children had a myelomeningocele and two were born with an anorectal anomaly (Malone 1990).
However, the Malone stoma, while within the surgical armamentarium, is not the innocuous, universally successful tool, suggested by some, with 69% of Hutson’s group having recurrent soiling after the formation of the access port, and there are a number of potential complications (King 2005).
Hutson’s group from Melbourne published a report of late follow-up of 56 children with a Malone stoma for the management of constipation, with only an 81% success rate, with 71% having symptoms at some stage during the course of using the washouts (King 2005), Complications were common in the use of the Malone stoma as shown in Hutson’s paper (King 2005), including Granulation tissue (79%), Anxiety about the Ace (50%), Stomal infection (43%), Stomal leakage in at least 38%, Embarrassment (36%), Dislike (29%), Stomal pain (26%), Stomal stenosis (19%), Behaviour disturbance (17%), stomal prolapse (14%), Stomal bleeding (14%), Limited activity (10%), Weight loss (5%), Perforation (5%). Poirier et al. recorded their complications of the Malone stoma for 18 patients, with only 78% success, they had 10 patients with 12 complications, including two with appendix perforation (Poirier 2007). Herndon et al. had a short mean follow-up with 13 stomal revisions in 117 patients and major complications such as caecal volvulus, small bowel obstruction, shunt infection but more importantly four patients who elected to not use the stoma due to intractable cramping with irrigation, which is a common complaint (Herndon 2004).
6. Timing of surgery for constipation
The argument that surgery should only be used after extensive medical management is trite at best. Surgery should be part of a package of options that is considered in all conditions that may require an operation. Obesity, for instance, should never require surgery, yet surgery is widely used to treat obese patients. Also, there are many procedures where surgery and medicine provide complementary options that the patient may prefer one over the other. There is argument that a failure to intervene surgically, and continue with unsuccessful medical treatment in the light of a clear indication for surgery, resulting in a long period of psychologic damage, is unacceptably deleterious – there is a balance that needs to be reached and a reasonable balance was achieved with this boy.
Many support the view that delaying appropriate treatment can have a devastating impact. Hutson, writing from the Royal Children’s in Melbourne maintains that 30% of children fail conventional medical management of constipation and develop long-term symptoms. His team go on to claim that “as 90% of faecal incontinence episodes are due to chronic constipation, there may be devastating effects on children’s emotional and social development” (King 2005; Southwell 2005). Coccorullo et al. also highlight the devastating impact of chronic constipation suggesting that the condition significantly interferes with a child’s emotional growth and development (Coccorullo 2009).
There have been several publications that support a regime that identifies the nature of the pathology as the guide to the timing of the surgery, rather than just the outcome of a particular sequence of medical treatments in a particular order over a particular duration. To highlight the acceptability of a short time-frame from diagnosis to surgical treatment in the presence of appropriate pathology let us look at the timeframe in a number of studies. Al-Salem, operated on four patients for removal of their megarectum, one of whom had a barium study virtually the same as this boy, with his patients ranging in age from 1.3 to 7 years, with a mean 3.9 years (Al-Salem 2008). Woodward’s whose 10 patients had a permanent colostomy created ranged in age from 2 to 15 years, five of whom were less than 9 years (Woodward 2003).
Obviously, the patients at the lower end of the above mentioned ranges had not had extended periods of medical management, but they had a combination of medical and surgical care directed at the specifics of their pathology. In 2005, King et al. reported the formation of Malone stomas in patients as young as 3.1 years (King 2005), while Gera et al. reported on 15 cases having laparoscopic resection of the rectosigmoid as young as 7 months (Gera 2009).
Other centres internationally have used various options for the management of constipation and faecal incontinence at a young age. Herndon, for example, treated 127 children from as young as 2.9 years with the formation of an antegrade enema stoma (Herndon 2004), Lee et al. resected bowel in a nine year old (Lee 2002), and Godbole’s unit performed resections of dilated bowel in 22 boys and 7 girls aged 3.5 to 14 years (Godbole 2001). The group operated on by Keshtgar et al., although born with anorectal anomalies, had effectively an isolated megarectum as their residual anomaly for which surgery was performed at an age range of 2-15 years. Those with normal sphincter and no neuropathy becoming continent in 7 of the 9, despite the young age of some of the group (Keshtgar 2007).
Also, the duration of therapy before obviously varies as indicated by the young ages, and evidence from groups such as Godbole et al., who used stimulant laxatives for as little as one month before surgery (Godbole 2001).
The other side of the duration of medical care is the long period of unsuccessful medical management reflected in some studies, which could be construed as a failure of timely use of a surgical solution. Lee et al. consider that once the diagnosis of megarectum is made, operative intervention should not be delayed because conservative management not only fails but also may have significant psychological and physical morbidity (Lee 2002). Fioretti et al. argued that even children who may have originally had a psychogenic cause of the constipation, subsequent regression results in an organic evolution independent of the original disturbance, so that surgery must be resorted to, as well as neuropsychiatric treatment. (Fioretti 1978). In other studies the lack of intervention at an early age resulted in significant complications and long periods of unsuccessful treatment. For examples, Brown and Shorthouse had a 16 and 25 year old patient who had symptoms from early childhood (Brown 1997), and operated on these young people even in the presence of low preoperative sphincter pressures with the existence of an idiopathic megarectum (Brown 1997). Stabiles group had one of seven who had distal dilatation from early childhood (Stabile 1991) and seven of Gattuso et al.’s chronic idiopathic constipation patients and a megarectum had surgery, some having unsuccessful treatment for up to six years (Gattuso 1997). Likewise, Stabile et al. had a group of 7 adults, five of whom had a good result from surgery for megarectum, but not before they had a difference between the median age of diagnosis and the median age of treatment of 14 years (Stabile 1992), which seems unacceptable. Better definition of the pathology, as was applied in “the case” would enable the select few who require surgery to have the intervention without the need for lengthy periods of failed management.
O'Suilleabhain also notes, as we have in PNG (Dewan 2000; Hrabovszky 2002; Dewan 2004), that dilated bowel does not involute once it has reached a certain degree of dilatation, despite diversion by a colostomy, in some cases for several years (O'Suilleabhain 2001). Thus, resection is inevitably needed.
Lundy identifies the complications from neglected patients with dilated bowel segments, recording the case of a 25 year old who had perforation secondary to his megarectum (Lundy 2006).
The various other points to highlight the appropriateness of surgery are that patients in adult life will often have an idiopathic megarectum or megacolon that may have the onset of symptoms in childhood or adulthood (Stabile 1992), effectively having been under treated. O'Suilleabhain argued that surgery was appropriate after the patient had had six months of their conservative management (O'Suilleabhain 2001). Williams also mentioned the common feature of onset in childhood, but that there can be delayed onset (Williams 2000), and Staiano demonstrated the failure of conservative treatment in a group of 62 children, with 63% still suffering constipation at the end of a five year study (Staiano 1994). It is staggering to think that medical management failure is so tolerated.
7. Outcome for megarectum surgery
The surgical literature clearly shows that surgery for a megarectum is successful, both when there is an underlying anorectal anomaly (Hrabovszky 2002; Keshtgar 2007; Levitt 2010) and when there is idiopathic constipation associated with a megarectum (Williams 2000; Godbole 2001; Al-Salem 2008; Levitt 2009).
Al-Salem, A. H. (2008). "Congenital segmental dilatation of the rectosigmoid colon: a forgotten cause of constipation." Journal of Pediatric Surgical specialties 2(2): 20-22.
Al-Salem, A. H., Grant, C. (1990). "Segmental dilatation of the colon." Disease of the Colon and Rectum 33: 515-518.
Amano, S., Tsukamoto, Y., Sakurai, A., Funada, M., Koike, H., Honzumi, M., Suzuki, H. (1985). "Pathophysiology and treatment of idiopathic chronic constipation with megarectum in children." Nippon Geka Gakkai Zasshi 86(9): 1273-1276.
Aslam, A., Grier, D.J., Duncan A.W., Spicer, R.D. (1998). "The role of magnetic resonance imaging in the preoperative assessment of anorectal anomalies." Pediatric Surgery International 14: 71-73.
Benninga, M. A., Buller, H.A., Heymans, H.S.A., Tygat, G.N.J., Taminiau, J.A.J.M. (1994). "Is encopresis always the result of constipation." Archives of disease in childhood 71: 186-193.
Brent, L., Stephens, F.D. (1976). "Primary rectal ectasia." Progress in Pediatric Surgery: 41-62.
Brown, S. R., Shorthouse, A.J. (1997). "Restorative proctocolectomy for idiopathic megarectum: postoperative recovery of hypotonic anal sphincters." Disease of the Colon and Rectum 40(5): 625-627.
Chiarioni, G., De Roberto, G., Mazzocchi, A., Morelli, A., Bassotti, C. (2005). "Manometric asessment of idiopathic megarectum in constipated children." World Journal of Gastroenterology 11(38): 6027-6030.
Coccorullo, P., Quitadamo, P., Martinelli, M., Staiano, M (2009). "Novel and alternative therapies for childhood constipation." Journal of Pediatric Gastroenterology and Nutrition 48(suppl 2): 104-106.
Croaker, G. D. H., Pearce, R., Li, J., Nahon, I., Javaid, A., Kecskes, Z. (2007). "Idiopathic slow transit constipation is rare." Pediatric Surgery International 23: 1153-1159.
Davenport, M., Bianchi, A. (1990). "Congenital intestinal atresia." British Journal of Hospital Medicine 44(3): 174-180.
de Lorijn, F., van Wijk, M.P., Reitsma, J.B., van Ginkel, R., Taminiau, J.A.J.M., Benninga, M.A. (2004). "Prognosis of constipation: clinical factors and colonic transit time." Archives of disease in childhood 89: 723-727.
Dewan, P. A., Elsworthy, E., Mathew, M., Poki, O., Khaw, S.L., Roberts, K., Catto-Smith, A. (2004). "Bowel imbrication in the management of anorectal anomalies." Pediatric Surgery International 20: 708 - 713.
Dewan, P. A., Hrabovszky, Z., Mathew, M. (2000). "Anorectoplasty in children in Papua New Guinea." PNG Medical Journal 43: 105-109.
Elawad, M. A., Sullivan, P.B. (2001). "Management of constipation in children with disabilities." Developmental medicine and child neurology 43: 829-832.
Etzioni, A., Benderly, A., Bar-Maor, J.A. (1980). "Segmental dilatation of the colon, andother cause of chronic constipation." Disease of the Colon and Rectum 23(8): 580-582.
Faucheron, J.-L., Dubreuil, A. (2000). "Rectal akinesia as a new cause of impaired defecation." Disease of the Colon and Rectum 43(11): 1545-1549.
Fioretti, G. P., Amici, G., Parmeggiani, P. (1978). "Megarectum in childhood." Minerva Chirurgia 33: 887-904.
Gattuso, J. M., Kamm, M.A., Talbot, I.C. (1997). "Pathology of idiopathic megarectum and megacolon." Gut 41: 252 - 257.
Gera, P., Jiwane, A., Jackson, R., Henry, G. (2009). Laparoscopic left colectomy in intractable constipation: an option. Nandi, Fiji.
Gladman, M. A., Dvorkin, L.S., Scott, M.S., Lunniss, P.J., Wiliams, N.S. (2006). "A novel technique to identifying patients with megarectum." Disease of the Colon and Rectum 50(5): 621-629.
Gladman, M. A., Williams, N.S., Scott, M.S., Ogunbiyi, O.A., Lunniss, P.J. (2005). "Medium-term results of vertical reduction rectoplasty and sigmoid colectomy." British Journal of Surgery 92(5): 624-630.
Godbole, P. P., Pinfield, A., Stringer, M.D. (2001). "Idiopathic Megarectum in Children." European Journal of Pediatric Surgery 11: 48-51.
Griffin, S. J., Parkinson, E.J, Malone, P.S.J. (2008). "Bowel management for Paediatric patients with faecel incontinence." Journal of Paediatric Urology 4: 387-392.
Halligan, S., Bartram, C.I. (1995). "The radiological investigation of constipation." Clinical Radiology 50: 429-435.
Hallows, M. R., Lander, A.D., Corkery, J.J. (2002). "Anterior resection for megarectosigmoid in congenital anorectal malformations." Journal of Pediatric Surgery 37(10): 1464-1466.
Hata, Y., Shinada, Y., Sasaki, F., Kameda, H., Saji, Y., Nishikawa, M., Uchino, J. (1986). "Surgical treatment of chronic constipation with megarectum in children." Nippon Geka Gakkai Zasshi 87(5): 531-535.
Herndon, C. D. A., Rink, R.C., Cain, M.P., Lerner, M., Kaefer, M., Yerkes, E., Casale, A.J. (2004). "In situ Malone antegarde continence enema in 127 patients: a 6-year experience." The Journal of Urology 172(172): 1689-1691.
Hrabovszky, Z., Dewan, P.A. (2002). "Revision anorectoplasty in the managment of anorectal anomalies." Pediatric Surgery International 18: 269-272.
Keshtgar, A. S., Ward, H.C., Richards, C., Graham, S. (2007). "Outcome of excision of megarectum in children with anorectal anomalies." Journal of Pediatric Surgery 42(1): 227-233.
King, S. K., Sutcliffe, J.R., Southwell, B.R., Chait, R.G., Hutson, J.M. (2005). "The antegrade continence enema successfully treats idiopathic slow-transit constipation." Journal of Pediatric Surgery 40: 1935-1940.
Kubota, A. (2008). Surgical therapies for intractable constipation in children. Pacific Association of Pediatric Surgery. Grand Tuton. : V99.
Langer, J., Caty, M., de la Torre-Mondragon, L., Gerogeson, K., Haddad, M., Hutson, J., Levitt, M., Pena, A. (2007). "IPEG Colorectal Panel." Journal of laparoendoscopic and advanced surgical techniques 17(1): 77-100.
Lee, S. L., DuBois, J.J., Montes-Garces, R.G., Inglis, K., Biediger, W. (2002). "Surgical management of chronic unremitting constipation and fecal incontinence associated with megarectum: a preliminary report." Journal of Pediatric Surgery 37(1): 76-79.
Levitt, M. A., Martin, C.A., Falcone, R.A., Pena, A. (2009). "Transanal rectosigmoid resection fro severe intracable idiopathic constipation." Journal of Pediatric Surgery 44: 1285-1291.
Levitt, M. A., Pena, A. (2010). "Pediatric fecal incontinence: a surgeon's perspective." Pediatrics in Review 31(3): 91-101.
Lundy, J. B., Gadacz, T.R. (2006). "Masive fecal impaction presenting with megarectum and perforation of a stercoral ulcer at the rectosigmoid junction." Southern Medical Journal 99(5): 525-527.
Malone, P. S., Ransley, P.G., Kiely, E.M. (1990). "Preliminary report: the antegrade continence enema." Lancet 336: 1217–1218.
Moore, B. G., Singgaram, C., Eckhoff, D.E., Gaumnitz, E., Starling, J.R. (1996). "Immunohistochemical evaluations of ultrashort segment Hirschsprung's." Disease of the Colon and Rectum 39: 817-822.
Nixon, H. H. (1967). "Megarectum in the older child." Proceedings of the Royal Society of Medicine 60(8): 801-803.
O'Suilleabhain, C. B., Anderson, J.H., McKee, R.F. Finlay, I.G. (2001). "Strategy for the surgical management of patients with idiopathic megarectum and megacolon." British Journal of Surgery 88: 1392-1396.
Oppe, T. E. (1967). "Megacolon and megarectum in older children." Proceedings of the Royal Society of Medicine 60(8): 803-805.
Pearl, R., Irish, M.S., Caty, M.G., Glick, P.L. (1998). "The approach to common abdominal diagnoses in infants and children." Pediatric Clinics of North America 45(6): 1287-1291.
Pena, A. (2002). "Coloin inertia disorders in Paediatrics." Current problems in Surgery(7): 661-732.
Poirier, M., Abcarian, H., Nelson, R., (2007). "Malone antegrade continent enema: an alternative to resection in severe defecation disorders." Disease of the Colon and Rectum 50(1): 22-28.
Preston, D. M., Lennard-Jones, J.E., Thomas, B.M. (1985). "Towards a radiological definition of idiopathic megacolon." Gastrointestinal radiology 10: 167-169.
Scarpa, M., Barollo, M., Keighley, M.R. (2005). "Ileostomy for constipation: long-term postoperative outcome." Colorectal Disease 7(3): 224-227.
Southwell, B., King, S.K., Hutson, J.M. (2005). "Chronic constipation in children: organic disorders are a major cause." Journal of Paediatrics and Child Health 41(1): 1-15.
Stabile, G., Kamm, M.A., Hawley, P.R., Lennard-Jones, J.E. (1991). "Colectomy for idiopathic megarectum and megacolon." Gut 32: 1538-1540.
Stabile, G., Kamm, M.A., Phillips, R.K.S. Hawley, P.R., Lennard-Jones, J.E. (1992). "Partial colectomy and coloanal anastomsis for idiopathic megarectum and megacolon." Disease of the Colon and Rectum 35(2): 158-162.
Staiano, M., Andreotti, M.R., Greco, L., Basile, P., Auricchio, S. (1994). "Long-term follow-up of children with chronic idiopathic constipation." Digestive Disease and Science 39(3): 561-564.
Swenson, O., Rathauser, F. (1959). "Segmental dilatation of the colon." American Journal of Surgery 97: 100-104.
van der Plas, R. N., Benninga, M.A., Staalman, C.R., Akkermans, L.M.A., Redekop, W.K., Taminiau, J.A., Buller, H.A. (2000). "Megarectum in constipation." Archives of Disease in Childhood 83: 52-28.
Weber, T. R., Tracy, T., Bailey, P.V., Lewis, J.E., Westfall, S. (1991). "Congenital diaphragmatic hernia beyond infancy." American Journal of Surgery 162(6): 643-646.
Williams, N. S., Fajobi, O.A., Lunniss, P.J., Scott, S.M., Eccersley, A.J., Ogunbiyi, O.A. (2000). "Vertical reduction rectoplasty: a new treatment for idiopathic megarectum." British Journal of Surgery 87(9): 1203-1208.
Woodward, M. N., Foley, P., Cusick, E.L. (2003). "Colostomy for treatment of functional constipation in children: a preliminary report." Journal of Pediatric Gastroenterology and Nutrition 38: 75-78.