Gastroesophageal Reflux and Other Disorders of Esophageal
Function
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To view a video of a laproscopic fundoplication procedure, click the
video icon. |
Fundoplication is currently either the first or the second most
commonly performed intra-abdominal procedure in infants and children in
most pediatric surgical centers. Since the 1970s, surgical correction
for gastroesophageal reflux (GER) has been performed by wrapping the
upper stomach around the esophagus either a partial (Thal, Boix-Ochoa,
Dor, Toupet) or complete (Nissen) fundoplication. In general, it
seems that the Nissen fundoplication is more effective in preventing
GER, but it may lead to difficulty swallowing and troublesome bloating
in some patients compared with a partial fundoplication, which seems not
as effective in preventing reflux. These symptoms after the Nissen
procedure seem to be minimized with use of an appropriate-sized
esophageal tube used as a “sizer” and division of the blood
vessels that attach the stomach to the spleen for sufficient
mobilization of the stomach.
PATHOPHYSIOLOGY
Physiologic vomiting is a benign variant of vomiting and is seen early
in life, usually after burping or feeding or when the infant is placed
in a lying down position. Physiologic vomiting in infancy does not
interfere with normal growth or development and rarely leads to other
complications. It is a self-limited process with symptoms resolving by
18 to 24 months of age in most patients. Usually, there is no treatment
indicated for these symptoms, and diagnostic evaluation rarely is
required.
In contrast to physiologic vomiting, GER can present as a single or
as multiple clinical conditions resulting in significant problems and
even near-fatal events. Many barriers exist to protect the patient
against reflux of gastric juice into the esophagus (Table1).
Table 1. Barriers to Gastroesophageal Reflux and Mechanism of
Failure
| Barriers to GGER |
Mechanisms of Failure |
| LES |
Inappropriate relaxation
Reduced LES length and pressure
Intrathoracic location
Abnormal smooth muscle function |
| Angle of His |
More obtuse angle (gastronomy)
Abnormal development |
| Length of intra-abdominal esophagus |
Length <3cm (adults) |
The two most important anatomic factors preventing GER appear to be
the length of the intra-abdominal esophagus and the angle at which the
esophagus enters into the stomach. Although no absolute effective
intra-abdominal esophageal length that prevents GER has been identified,
there is correlation between increased intra-abdominal esophageal length
and lack of GER.
It seems that an intra-abdominal length of 3 to 4.5cm in adults with
normal intra-abdominal pressure provides sufficient protection against
reflux most of the time. A length of 3cm seems sufficient in preventing
reflux in approximately half of adult patients, whereas a length of less
than 1cm results in reflux in most patients.
When the usual sharp angle at which the esophagus enters into the
stomach becomes flatter, GER is more prone to develop. On the other
hand, increase of this angle seems to inhibit reflux. The ability of
this angle to participate in prevention of GER may be diminished as a
result of abnormal development or may occur after gastrostomy tube
placement.
Components working against this barrier to reflux include the lower
esophageal muscles; the difference between abdominal and chest
pressures; and various naturally occurring substances which alter the
tone of the esophageal and stomach muscles. The lower esophagus has an
inner muscle layer, which is thickened. This thickened muscle layer
creates a high-pressure zone that can be measured. This muscular
thickening extends onto the stomach. This acts as a valve in the lowest
portion of the esophagus. The ability to prevent GER is directly
proportional to this valve effect and the length of this lower
musculature, provided that the muscle relaxes normally.
If relaxation of this muscle is excessive, GER can develop. Also,
malposition of the muscle, which can occur with a hiatal hernia in which
the area where the esophagus joins the stomach is up in the chest
instead of in the abdomen, or with abnormal development, results in loss
of the valve effect and contributes to development of GER. Lower
esophageal muscle relaxation occurs when the upper esophagus tries to
push food down towards the stomach, which is initiated by the swallowing
mechanism. If this normal relaxation is exaggerated and is unassociated
with swallowing, GER results.
Although the valve function is sometimes imperfect, it is usually
highly effective. Short muscle length, abnormal muscle function,
increased frequency of muscle relaxation, and muscle location within the
chest can contribute individually or in combination to GER.
CLINICAL MANIFESTATIONS
There are many ways that infants and children present with GER.
Persistent regurgitation is the most common clinical symptom of GER,
especially in young children and in neurologically impaired patients.
This excessive vomiting can result in inadequate nutritional intake and
can lead to growth failure.
Respiratory symptoms also are seen commonly in infants and children
secondary to GER. Identifying that GER is the reason for ongoing
respiratory symptoms can be difficult because the symptoms may be the
same as those often seen with other lung diseases. Chronic cough,
wheezing, choking, or near sudden infant death syndrome (SIDS) all can
be attributed to GER.
Acid within the esophagus causes the vocal cords and the small
airways of the lung to spasm, which clinically presents as apnea or
choking and can be mistaken for asthma. Irritation of the esophagus,
likely enhances this mechanism. Although major bleeding is a rare
presenting symptom, slow blood loss with iron deficiency anemia can
occur occasionally.
In pediatric patients, another presenting symptom is irritability
owing to pain. This discomfort can lead to crying in infants despite
consoling measures. Although small volumes of feedings in infants can
alleviate this discomfort briefly, it is not a lasting benefit. Pain is
a presenting symptom often in older children as well. As in adults, the
pain is usually located under the breast bone and is described as
heartburn. Whether in infants or older children, long-standing GER with
inflammation or irritation of the esophagus can lead to scarring and
narrowing of the esophagus. When a stricture (narrowing) develops, pain
and trouble swallowing are the two most commonly associated
symptoms.
Barrett’s esophagitis is a precancerous condition in which the
lining of the distal esophagus changes. In adults, it is believed to be
the result of chronic esophageal injury from gastric acid reflux. This
condition is rare in infants and children. When it does develop,
however, serious complications often result. In addition to the
increased risk for cancer in adults, approximately 50% of these patients
develop an esophageal stricture, and many develop ulcerations.
Barrett’s esophagitis can be seen in the older child and teenager
as a result of long-standing GER. For this reason, looking into the
esophagus and sampling the lower lining may be more appropriate in the
evaluation of the older patient when compared with the infant. When
Barrett’s esophagitis is documented in the child, it usually can
be managed successfully with an anti-reflux operation.
DIAGNOSTIC EVALUATION
When GER has been entertained as the cause of the patient’s
symptoms, diagnostic evaluation should be initiated. Among the possible
studies used for evaluation of GER are the upper gastrointestinal (GI)
contrast study, 24-hour pH monitoring, esophageal manometry (pressure
monitoring), upper GI endoscopy (direct telescopic visualization), and
nuclear medicine gastric emptying studies.
The use of these studies varies according to the medical center in
which the patient is seen. Although some surgeons and
gastroenterologists believe that all patients should undergo a gastric
emptying study preoperatively, many do not because gastric emptying is
thought to improve with anti-reflux surgery owing to a decrease in the
capacity of the stomach. For this reason, it can be difficult to
interpret which patient with delayed gastric emptying on a preoperative
study is benefited by a gastric emptying operation at the time of
anti-reflux surgery. Esophageal manometry is not used routinely in
children but is beneficial when there is concern about a motility
problem in the esophagus or abnormally high pressure at the bottom of
the esophagus (achalasia).
The 24-hour pH monitoring test is an objective way to document the
presence or absence of GER. This test can be particularly helpful in
patients in whom the history is unclear or confusing, such as a patient
presenting with respiratory symptoms only. For an accurate result, it is
important to cease all anti-reflux medication before the study.
Proton-pump inhibitors should be withheld for 7 days, and histamine
receptor blockers should not be given for 72 hours before the
examination. The patient often needs to be admitted for this test.
An upper GI contrast study is the most frequently used study for
initial evaluation of GER. Although not as sensitive as 24-hour pH
monitoring, it can be performed on an outpatient basis. In addition, if
reflux is documented on this study, and if the patient’s symptoms
are consistent with this diagnosis, 24-hour pH monitoring may not be
necessary in all circumstances.
If the upper GI series is normal, however, and the suspicion of
reflux persists, 24-hour pH monitoring is recommended. In centers where
the upper GI series and 24-hour pH monitoring are employed routinely for
diagnosis, the upper GI series is used primarily to document anatomic
abnormalities of the stomach and first part of the intestine or gastric
outlet obstruction (blockage of the outlet of the stomach) that might be
contributing to the patient’s symptoms. The upper GI series
provides a crude evaluation of whether or not there is normal or delayed
gastric emptying.
Direct visualization of the esophagus and stomach is used in some
centers for the routine evaluation of infants and children with
suspected GER. When endoscopy is used, biopsy is performed to document
inflammation or irritation of the esophagus and to exclude cancer or
precancerous lesions in older children. Symptoms of bloody vomiting or
irritability in infants or trouble swallowing with or without heartburn
in infants and children should prompt a direct look into the esophagus
and stomach with a telescope (endoscopy).
The presence of delayed gastric emptying is assessed best
preoperatively using a nuclear scan with radioactive eggs or milk.
Neurologically impaired children with GER have been shown to have
delayed gastric emptying more often than neurologically normal children.
The benefit and complication rates for these patients undergoing
emptying procedures at the time of anti-reflux surgery are unclear. In
addition to assessing delayed gastric emptying, the technetium scan is
able to help evaluate possible aspiration into the lungs.
TREATMENT
Medical Management
Initial treatment for “garden variety” GER is usually
medical. Initial non-operative treatment in infants includes upright
positioning and thickened feedings with or without the addition of
medicine that helps the stomach empty (Reglan). Most infants respond to
medical treatment and do not require a surgical procedure. H 2-blockers,
such as cimetidine or ranitidine, also may improve the success of
non-operative therapy. A proton-pump inhibitor (e.g., omeprazole) has
revolutionized medical therapy and is the most effective agent for
non-operative treatment of this disorder.
Indications for operation without initially attempting medical
therapy include the presence of precancerous lesions, narrowings or
strictures, a near-fatal (SIDS) event, and, in some centers, infants who
stop breathing momentarily enough times to lower their heart rates. If
medical therapy is employed, failure of medical therapy constitutes an
indication for an operation. It is unclear what is the appropriate time
between initiation of medical therapy and consideration of surgical
intervention for failure of medical therapy. In infants, it seems
reasonable to use medical therapy for several months before operation.
In older children, the risk of complications is greater, and medical
therapy probably should not be employed more than 6 to 8 weeks.
Surgical Procedures
As previously mentioned, many operations have been used in the surgical
management of infants and children with GER, including the Nissen
fundoplication, the anterior (Thal) fundoplication, the Boix-Ochoa
procedure, and the posterior (Toupet) fundoplication. The Nissen
procedure is the most commonly used fundoplication in the United States.
The Nissen fundoplication is a full 360-degree wrap, whereas the Thal
fundoplication is an anterior 270-degree partial wrap. Cited
disadvantages of the Nissen procedure are the inability to vomit,
trouble with swallowing, and the development of stomach gas bloating.
Potential gas bloat seems to occur less frequently in children than in
adults. Although the Thal fundoplication has a higher incidence of
recurrent reflux compared with the Nissen fundoplication, it does not
have the associated postoperative problems of swallowing difficulties
and bloating. The Nissen fundoplication lends itself best to the
laparoscopic approach and is the most commonly performed laparoscopic
fundoplication in the world.
When either the Nissen or the Thal operation is performed open or
laparoscopically, the results are good, especially in the short-term.
Because the laparoscopic approach for either operation has not been used
extensively for more than 5 years as of this writing, long-term results
with the laparoscopic approach are not known. A recurrence rate of 10%
for the Nissen procedure and 15% to 20% for the Thal operation has been
found in most large series performed in the open fashion. The most
common cause for recurrence with the Nissen fundoplication is migration
of the wrap up into the chest from the abdomen followed by breakdown of
the wrap. Symptoms of recurrent reflux are more common in patients with
neurologic impairment than in patients who are neurologically normal.
Postoperative intestinal obstruction, which may occur in 15% to 25% of
cases after an open fundoplication, is rare following the laparoscopic
operation.
A common scenario for pediatric surgeons is a child, usually
neurologically impaired, in need of a gastrostomy for feeding and
nutrition. Decisions of whether or not to perform an evaluation for GER
and how to perform this evaluation vary from surgeon to surgeon. At the
least, most surgeons obtain an upper GI series to evaluate for the
presence or absence of GER. In a patient with symptoms of reflux, a
fundoplication should probably be performed. In a patient without
clinical symptoms of reflux and a normal upper GI series and a normal pH
probe, gastrostomy alone may be an option. However it should be
recognized that placement of a gastrostomy to be likely increases the
incidence of GER. If reflux is noted on either the upper GI series or
the pH study, and especially if the patient is neurologically impaired,
a fundoplication in addition to the gastrostomy is recommended. In
neurologically impaired patients, some surgeons also routinely order a
gastric emptying study to evaluate whether or not delayed gastric
emptying is present and, if so, perform a gastric emptying procedure.
Some neurologically impaired patients with GER are managed initially
by radiographic placement of a nasojejunal tube for enteral
alimentation. If a gastrostomy has been created previously, and GER has
developed, a gastrojejunal tube placed via the gastrostomy stoma also
can be used. The main disadvantage of this approach is the frequent
dislodgment of these tubes and recurrent trips to the fluoroscopy suite
for reinsertion. In addition, underlying reflux and the basic pathologic
conditions remain untreated.
Two special circumstances that merit thoughtful evaluation before
fundoplication are the patient who previously has required repair of
esophageal atresia and the patient, usually neurologically impaired, who
has failed two or three prior fundoplications. In the patient with
previous esophageal atresia, there is underlying esophageal motility
problems, and this may be aggravated by a 360-degree wrap. For this
reason, a partial wrap may be recommended in these patients. In the
patient who already has failed two or three previous fundoplications
mechanically separating the esophagus and stomach is an attractive
alternative approach.
Technique
Nissen Fundoplication
The Nissen fundoplication can be performed either laparoscopically or
open. The laparoscopic approach is being used more frequently because of
its perceived advantages of reduced discomfort and length of
hospitalization and earlier return to routine activities, such as school
or sports in older children. Whether performed laparoscopically or open,
the operation is done in a similar fashion. In the open operation,
either an incision from the bottom of the breast bone toward the belly
button or an incision below the left ribs is used. With the laparoscopic
operation, five small (3 or 5 mm) incisions are employed (Figure 1).

Figure 1: The incisions (arrows) and the gastrostomy button that
was placed at the time of the laparoscopic fundoplication in this
infant. |
Whether performed in an open or laparoscopic
fashion, the blood vessels connecting the stomach and spleen are
divided. After division of these blood vessels, the esophagus is
identified and an adequate length of intra-abdominal esophagus is
obtained. A hiatal hernia (a “too large” opening in the
diaphragm through which the esophagus passes) may be present that
requires one or two sutures for closure. If used, an
appropriate-sized dilator is directed through the mouth and into the
stomach to help make sure that the wrap is not too tight.
Figure 2: A) The stomach is pulled downward to straighten the
esophagus. Note the suture that was used to close the extra opening in
the diaphragm behind the esophagus. B) The two halves of the
fundoplication (stomach wrap) is shown by the arrows. |
The top of the stomach is brought behind the
esophagus, and the 360-degree wrap is completed silk sutures (Figure 2,
3 and 4). If required, a gastrostomy can be placed at the time of
the fundoplication (Figure 6).
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| Figure 3: The partially completed wrap or fundoplication is shown.
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Figure 4: The fundoplication is completed. Note how the stomach is
wrapped around the esophagus and sewn back to itself to complete a
circle |
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Thal Fundoplication

Figure 5: The open Thal fundoplication. A, B) The attachments of
the left liver are taken down from the underside of the diaphragm. The
liver can be turned downward and to the right, exposing the esophagus
where it comes through the diaphragm. C) A dissector is passed behind
the esophagus. D) Dacron tape is passed about the esophagus when the
dissector is withdrawn, allowing the esophagus to be pulled downward. An
appropriate length of esophagus is exposed for placement in the intra-
abdominal position. E, F) A suture is used to bring the diaphragm muscle
behind the esophagus together. G, H, I, J) The top of the stomach is
brought up and sutured to the front half of the esophagus. A continuous
suture is used, progressively suturing the stomach to the esophagus. The
continuous suture is completed and tied. K) This method creates an
inverted U-like suture line. The dashed lines show the position of the
esophagus in relation to the stomach when the wrap is complete. A
nasogastric tube is positioned during the procedure. The tube may be
removed immediately afterward or may be left until the following day,
when it is removed. Feedings are then begun. (From Ashcraft KA:
Gastroesophageal reflux. In Ashcraft KW, Holder TH [eds]: Pediatric
Surgery, 3rd ed. Philadelphia, WB Saunders, 2000.) |
This fundoplication also can be performed using an open or the
laparoscopic technique. A hiatal hernia, if present, is repaired. The
anterior wrap is accomplished by sewing the stomach adjacent to the left
side of the esophagus to the esophagus. The suture line is continued
across the esophagus as the stomach is folded onto the lower esophagus
toward the right. When the right side of the esophagus is reached, the
suture line is turned downward to sew the folded over stomach wall and
the right lower esophagus (Figure 5).
OTHER DISORDERS OF ESOPHAGEAL FUNCTION
Anatomically, the esophagus is made up of two types of muscle fibers:
the upper one third of the esophagus is composed of striated muscle, and
the remaining lower portion is composed of smooth muscle. Functionally,
there are three zones to the esophagus: the upper esophageal sphincter
(UES), the body, and the lower esophageal sphincter (LES). Swallowing is
a complex series of coordinated contractions and relaxations of these
muscle fibers in these physiologic areas. When this does not occur in a
coordinated fashion an esophageal motility disorder occurs.
Compared with GER, esophageal motility disorders are rare in
children. These disorders include achalasia, diffuse esophageal spasm,
and scleroderma. The diagnosis of these disorders is based on clinical
suspicion, upper GI contrast study, manometry, and, occasionally, pH
monitoring. Dysphagia or difficulty swallowing is usually a prominent
symptom with these esophageal motor disorders.
Achalasia
Achalasia is a motor disorder usually affecting the entire length of the
esophagus. Although the cause of achalasia is unclear, approximately 10%
of cases occur in children. Even though this disease is relatively
uncommon in children, it seems to be increasing in frequency. Two thirds
of affected children are boys. Whether the condition is being diagnosed
at an earlier age or there is a true increase in its incidence is
unclear. This disorder is caused by failure of the LES to relax and
failure of the esophagus to advance food into the stomach.

Figure 6: Chest x-ray shows an air-fluid level in the middle of
the chest (arrow) in a patient with achalasia. |
Most children with achalasia present with symptoms of several
months’ to several years’ duration consisting typically of
vomiting of undigested food, severe bad breath, and significant
dysphagia (difficulty swallowing). These children also may have
prolonged coughing spells after meals. Weight loss is common because the
nutritional status is affected. Children often complain of pain behind
their breastbone or burning secondary to esophageal irritation from food
that is stuck. Usually there is a delay in establishing a diagnosis. A
chest x-ray may show a widening in the middle of the chest and a fluid
level in the esophagus when the child is sitting or standing. (Figure
6A).
The diagnosis usually is suspected after an upper GI series shows
narrowing at the LES with a large esophagus above it (bird beak
deformity) (Figure 6B)
In addition, there is no relaxation of the LES on UGI study.
The diagnosis is confirmed by manometric (pressure) evaluation revealing
an elevated resting pressure in the esophagus without evidence of
relaxation of the LES (Figure 7). There also is absence of coordinated
contractions in the esophagus.

Figure 7: Manometric study shows a markedly elevated resting
pressure in the esophagus without evidence of relaxation at the LES in a
patient with achalasia. |
The ideal treatment of patients with achalasia is controversial. In
1971, Van Trapp introduced the concept of balloon dilation of the
narrowed LES for adult patients. Balloon dilation is used in children
but seems to be less successful in relieving symptoms than in adults.
The incidence of post-dilation GER may be higher in children, and the
procedure is technically more difficult in younger patients. Other
approaches for relief of symptoms include injection of botulinum toxin
into the LES. Although this technique has gained popularity in more
recent years, its effectiveness usually lasts only a few months. Medical
management is also not successful. Because non-operative therapy is
rarely successful, especially for a prolonged period, most children with
achalasia require an operation for long-term relief.
In 1913, Heller described an operation for relief of symptoms of
achalasia. With advancements in minimally invasive surgery, this
procedure has become an attractive approach for surgical management.
Although esophagomyotomy (Heller’s procedure) has been reported
using either the laparoscopic (through the abdomen) or the thorascopic
(through the chest) route, the laparoscopic route is now favored. There
are many reports in adults describing the effectiveness of a
laparoscopic Heller myotomy for relief of symptoms resulting from
achalasia, but the experience in the pediatric literature is not nearly
as large.
The most controversial aspect of performing a laparoscopic
esophagomyotomy in a child is whether or not an anti-reflux procedure
should be added. This controversy also exists in the adult literature.
Most adult surgeons favor the performance of an anti-reflux operation
because it is relatively easy to accomplish and prevents the need for a
second procedure if GER develops. In most children a fundoplication is
not performed.
Figure 8: Intraoperative photograph depicts the completed
esophagomyotomy to a length of 8cm. The muscle fibers are widely
separated (open arrows), and the anterior vagus nerve crosses the
submucosa (solid arrow). |
Esophagomyotomy
For the laparoscopic esophageal myotomy, the placement of the incisions
is similar to that used for fundoplication (Figure 1). Identical
instruments are employed for a laparoscopic esophageal myotomy as for a
laparoscopic fundoplication.
With an operative plan of myotomy and anterior fundoplication, the
initial step is to divide the blood vessels connecting the stomach and
spleen in order to free the upper stomach. Next, the anesthesiologist is
asked to introduce an appropriate-sized dilator through the mouth and
into the stomach to open up the lower esophagus. Some surgeons also use
esophagoscopy to do this.
The first step in performing the myotomy is careful separation of the
esophageal muscle fibers 1 to 2 cm above the LES (Figure 8). The muscle
fibers are gently hooked and pulled away from the inner esophageal
linings (Figure 12). The myotomy usually is extended upward to
above the opening in the diaphragm where the esophagus enters the
abdomen. With a myotomy to this level, the length is 6 to 8 cm (Figure
13). If a longer esophageal myotomy is desired, pulling the esophagus
downward may help. A Penrose drain or umbilical tape can be placed
around the esophagus and the esophagus retracted downward for this
purpose. The myotomy is also continued for 1-2 cm onto the
stomach. If there is concern about the completeness of the myotomy, the
dilator can be removed and direct endoscopic visualization performed to
confirm that an adequate myotomy has been accomplished. If required, the
esophagomyotomy can be extended further onto the stomach. If performed,
a fundoplication is created.
The postoperative management varies from surgeon to surgeon. Some
proceed with a liquid diet the evening of the operation with discharge
the following morning. Others prefer to decompress the stomach overnight
with a tube placed through the nose and into the stomach. Some may
prefer to perform an UGI contrast study the following morning to
ensure there is no evidence of perforation (a hole) followed by a liquid
diet if no perforation is noted on the contrast study. A mechanical soft
diet usually is employed for several days, after which a regular diet
may be initiated. There is usually no need for additional x-rays unless
symptoms recur.
Diffuse Esophageal Spasm
Diffuse esophageal spasm is even less common than achalasia. Symptoms of
this disorder are similar to symptoms seen with achalasia. Patients
present with a long-standing history of swallowing problems,
regurgitation of undigested food, bad breath, and weight loss. Children
also may complain of chest pain under the breast bone of a pressure or
burning nature. Lung symptoms, such as pneumonia or asthma, also may be
present. Cold beverages may make these symptoms worse. The diagnosis
usually is made with pressure measurements (manometry) and an upper GI
study. On UGI study, areas of contraction of the esophagus are seen,
giving the appearance of a distorted corkscrew or rosary beads. On
manometry, there are repeated prolonged high pressure areas in the lower
one half to two thirds of the esophagus with relaxation of the LES.
Little is written on the management of children with this disorder.
In adults, there have been reports of successful treatment with
injection of botulinum toxin in the LES, psychosomatic treatment with
antidepressants, and ingestion of peppermint oil along with
nitroglycerin. Balloon dilation and surgical myotomy also have been
employed with intermittent success. Neither of these invasive approaches
is totally satisfactory.
Scleroderma
Scleroderma is a connective tissue disorder in which esophageal motor
dysfunction is only one part of the syndrome. Although esophageal
dysmotility can occur in other connective tissue disorders, it is most
common in scleroderma and is found eventually in 80% of patients. In
addition, delayed gastric emptying also is often seen in these patients.
The development of esophageal dysfunction in patients with scleroderma
also has been found to correlate with the development of lung disease.
The abnormality consists of scarring and wasting of the esophageal
smooth muscle. This results in poor contractions of the esophagus and in
decreased lower esophageal sphincter tone, leading to GER, narrowing of
the esophagus (stricture), aspiration, malnutrition, and growth failure.
In children with scleroderma, GI involvement seems more frequent than
the clinical symptoms indicate. An active diagnostic evaluation for GI
dysmotility and GER is necessary to detect these complications.
There is no effective treatment for this disorder. An anti-reflux
procedure can be performed if the symptoms of GER cannot be managed
medically. If the esophageal length is shortened, an esophageal
lengthening procedure may be necessary. A partial wrap may be more
appropriate than a complete wrap because of the potential for
obstruction at the gastroesophageal junction with the latter operation.
SUGGESTED READINGS
Chung DH, Georgeson KW, Winters DC: Laparoscopic gastrostomy as
adjunctive procedure to laparoscopic fundoplication in children.
Surg Endosc 10:1106-1110, 1999.
The authors describe their technique for laparoscopic fundoplication and
the U-stitch technique for laparoscopic gastrostomy.
Dalla Vecchia LK, Grosfeld JL, West KW, et al: Reoperation after
Nissen fundoplication in children with gastro esophageal reflux:
Experience with 130 patients. Ann Surg 226:315-321, 1997.
In a series of 130 patients, of whom 78% were neurologically impaired, a
breakdown of the fundoplication occurred in 42%, and migration of the
wrap through a hiatal hernia occurred in 30%. Both problems developed in
21% of the patients.
Danielson PD, Emmens RW: Esophagogastric disconnection for
gastroesophageal reflux in children with severe neurological impairment.
J Pediatr Surg 34:84-86, 1999.
The authors describe 27 patients who required esophagogas- tric
disconnection. All patients were severely neurologically impaired. This
procedure was performed primarily in 26 of the patients.
Fonkalsrud ER, Ashcraft KW, Coran AG, et al: Surgical treatment of
gastroesophageal reflux in children: A combined hospital study of 7,467
patients. Pediatrics 101:467- 468, 1998.
This is a retrospective review of the authors’ combined clinical
experience with surgical treatment of symptomatic GER in children over
20 years. This is the largest cohort of patients who have undergone
surgical treatment of GER.
Hurwitz M, Bahar RJ, Ament ME, et al: Evaluation of the use of
botulinum toxin in children with achalasia. J Pediatr Gastroenterol
Nutr 30:509-514, 2000.
Botulinum toxin was used in 23 children. Because many of the children
eventually will require an additional procedure (dilation or
esophagomyotomy), the authors recommend this technique only in children
who are poor candidates for either dilation or surgery.
Patti MG, Albanese CT, Holcomb GW III, et al: Laparo- scopic Heller
myotomy and Dor fundoplication for esophageal achalasia in children.
J Pediatr Surg 36:1248-1251, 2001.
A small series of children underwent laparoscopic esophagomyotomy and
anterior fundoplication for achalasia. All patients were discharged
within 2 days of the procedure.
Patti MG, Pellegrini CA, Horgan S, et al: Minimally invasive surgery
for achalasia: An 8- year experience with 168 patients. Ann
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In this large series of 168 adults, the authors describe the evolution
in their surgical technique and their clinical results.
Poirier NC, Taillefor R, Topart P, et al: Antireflux operations in
patients with scleroderma. Ann Thorac Surg 58:66-72, 1994.
Fourteen patients with scleroderma underwent an antireflux procedure.
Reflux symptoms were relieved in 10 of the patients through a variety of
operations. Antireflux procedures in patients with scleroderma can
palliate esophageal injury caused by GER without jeopardizing esophageal
function.
Rudolph CD, Mazur LJ, Liptak GS, et al: Guidelines for evaluation and
treatment of gastroesophageal reflux in infants and children:
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32(Suppl 2):S1-31, 2001.
This article describes clinical practice guidelines for the management
of GER in children that were formulated by the GER Guideline Committee
of the North American Society for Pediatric Gastroenterology and
Nutrition. The guidelines provide recommendations for management by the
primary care provider, including evaluation, initial treatment,
follow-up management, and indications for consultation by a specialist.
It also provides recommendations for management by the pediatric
gastroenterologist. These guidelines have been endorsed by the American
Academy of Pediatrics.
Snyder CL, Ramachandran V, Kennedy AP, et al: Efficacy of partial
wrap fundoplication for gastroesophageal reflux after repair of
esophageal atresia. J Pediatr Surg 32:1089-1091, 1997.
The authors review their experience with 59 children who required Thal
fundoplication for surgical management of GER after correction of
esophageal atresia and tracheoesophageal fistula. In this series, 15% of
the children required reoperation compared with a failure rate of 4.3%
in the authors’ experience of patients without esophageal atresia.
Storr M, Allescher HD, Classen M: Current concepts on
pathophysiology, diagnosis and treatment of diffuse esophageal spasm.
Drugs 61:579-591, 2001.
This article describes current concepts regarding the physiology,
diagnosis, and treatment of patients with diffuse esophageal spasm.
Articles and graphics adapted from O'Neill: Principles of Pediatric
Surgery. © 2003, Elsevier.
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