Gastroesophageal Reflux With Protein-Losing Enteropathy and Finger Clubbing John J.
Herbst, MD; Dale G. Johnson, MD; Miguel
\s=b\ Finger clubbing, protein-losing opathy, and iron deficiency were
mented in three children with
enterdocusevere
gastroesophageal reflux. One patient had Sandifer syndrome and the other two had the rumination syndrome. In each case, surgical repair of the gastroesophageal reflux resulted in immediate clearing of signs of the Sandifer syndrome, gastroesophageal reflux, and anemia and the return of serum protein levels to normal. There was definite regression of the finger clubbing during the ensuing year. It is suggested that finger clubbing, proteinlosing enteropathy, Sandifer syndrome, and rumination be viewed as parts of an extended syndrome of unusual presentations of gastroesophageal reflux. (Am J Dis Child 130:1256-1258, 1976) reflux with or without hiatal hernia has been associated with a number of complica¬ tions, including failure to thrive,
Gastroesophageal
anemia, aspiration pneumonitis,
se¬
esophagitis, and stricture.' We describe three children with hiatal hernia, severe reflux esophagitis, iron vere
-
deficiency anemia, finger clubbing,
From the departments of pediatrics (Drs Herbst and Oliveros) and surgery (Dr Johnson), University of Utah Medical Center, Salt Lake
City. Reprint requests to Department of Pediatrics, University of Utah Medical Center, 50 N Medical Dr, Salt Lake City, UT 84112 (Dr Herbst).
A.
Oliveros,
MD
and protein-losing enteropathy. We believe these are the first case reports of such associated conditions. The only previous association of hiatal hernia and clubbing of the fingers in the literature is a brief reference to it in two children by Botha. Protein-losing enteropathy is a relatively common phenomenon associated with many disorders of the gastrointestinal tract, but carcinoma of the esophagus is the only disease primarily affecting the esophagus with which it has been related previously. '
REPORT OF CASES Three children with finger clubbing, protein-losing enteropathy, and severe gastroesophageal reflux were studied. The clubbing was quantitated by measuring the distal phalangeal-interphalangeal ratio as proposed by Waring et al.' Proteinlosing enteropathy was diagnosed by measurement of fecal excretion of radioac¬
tive chromium ("Cr) following intravenous injection of a bolus of "''Cr-labeled albu¬ min."' Case 1.—A 4V2-year-old boy was referred to us because of microcytic anemia unre¬ sponsive to ferrous sulfate therapy. He had a history of frequent "spitting" since birth. During the 18 months prior to referral, he vomited at least once every other day. The emesis was frequently blood-stained, and he exhibited a tendency to cock his head, often assuming this peculiar posture while recumbent (Fig 1). He complained of pain in the upper sternal area that sometimes awoke him at night, and he often obtained
by drinking large amounts of milk. a pale, irritable child, who frequently cocked his head to one side. His height and weight were at the 50th percen¬ tile for age. Clubbing of the fingers was evident and the ratio of the distal phalangeal-interphalangeal depth was 1.030 (nor¬ mal less than 0.985). The hemoglobin level was 6.7 gm/100 ml and the hematocrit reading was 25.5%. Serum iron level was 6/ig/100 ml, and serum iron-binding capac¬ ity 221ug/100 ml. Total serum protein level was 4.3 gm/100 ml, and albumin 2.3 gm/ 100 ml. After intravenous injection of ''Crrelief He
was
=
labeled
albumin,
five-day
stool
21'??
was
detected in
a
sample (normal less than 2%). A cine-esophagram showed a large hiatal hernia and gross gastroesophageal reflux up to the superior esophageal sphincter, and reflux seemed to occur just prior to the act of head cocking. X-ray films of the chest and small and large bowel
were
normal,
=
as was a
small bowel
biopsy specimen. The symptoms, anemia, and serum protein concentrations did not improve following oral ferrous sulfate therapy and institution of intensive medi¬ cal treatment of the gastroesophageal reflux.
Esophagoscopy at the time of operation showed severe reflux esophagitis without stricture. At operation, the entire thick¬ ness of the esophagus was inflamed and edematous, with considerable local mediastinal reaction. A combined Borema and Nisson repair of the hiatal hernia and gastroesophageal reflux was performed. Postoperatively, the head cocking disap¬ peared almost completely within three days. The serum protein levels returned to
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Fig 1.—Head-cocking
trait
(case 1).
Fig 2.—Esophagram demonstrating
Fig
3.—Clubbed
massive reflux and
(case 2).
Features Quantitation of
Age,
hiatal hernia
finger (case 3).
Major Clinical
Case
large
yr 4'/2 5'/2 11
Hemoglobin, gm/100 ml 6.7 6.6 7.6
Albumin,
gm/100
Fecal* s'Cr 21.0 11.6 2.8
ml
2.3 2.4 3.2
Finger Clubbing!
Preoperatively
Postoperatively
Syndrome
1.030 1.102 1.020
0.966
Sandifer Rumination Rumination
"Percent of intravenously administered bolus ofs Cr-labeled albumin detected In 0.985. fRatlo of distal phalangeal depth to interphalangeal depth.
five-day
0.982 stool
sample.
=
normal within two weeks, and his anemia cleared in six weeks with oral ferrous sulfate therapy. Within nine months, the ratio of the distal interphalangeal depth to the interphalangeal depth had returned to normal (0.966), and clubbing was not noted
clinically (Table). Case 2.—A 5y2-year-old white boy was referred to us with a severe microcytic anemia and a hemoglobin level of 6.6 gm/ 100 ml. He had vomited frequently since birth and had been taking ferrous sulfate since 9 months of age for chronic iron deficiency anemia. Two years previously, a hiatal hernia with free gastroesophageal reflux had been diagnosed (Fig 2), and positional therapy was initiated. He con¬ tinued to vomit at least three times a week; however, no history of head cocking was obtained. Physical examination showed a thin boy in the 50th growth percentile for
height and the 30th growth percentile for weight. He had definite clubbing of the fingers and toes, the ratio of the distal phalangeal depth to the interphalangeal depth being 1.102. Total serum protein level was only 5.2 gm/100 ml. Results of liver function tests were normal, and stool guaiac determina¬
tions were positive. X-ray studies revealed a normal chest, a large hiatal hernia, and free gastroesophageal reflux. Within one week of operative repair of the gastro¬ esophageal reflux, the intestinal blood loss stopped, the serum protein levels returned to normal, and emesis and other symptoms of the rumination syndrome ceased. Over the next nine months, the finger clubbing improved and the ratio of the depth of the distal phalangeal depth to the interphalan¬ geal depth decreased from 1.020 preoperatively to 0.982.
Case 3.—An 11-year-old mentally re¬ tarded boy was referred to us because of emesis, which had persisted since infancy. In recent months, it had increased in severity and he started to show the typical clinical signs of the rumination syndrome. Findings from physical examination were remarkable only in that he had finger clubbing (Fig 3). He had an iron deficiency anemia with a hemoglobin level of 7.6 gm/ 100 ml. Stool guaiac tests were positive. Serum protein level was 5.8 gm/100 ml and the albumin level was 3.2 gm/100 ml. He had radiological evidence of a large hiatal hernia and severe gastroesophageal reflux. Roentgenograms of the small and large bowel and a small bowel biopsy specimen were all normal. The patient's anemia improved after oral iron therapy, but the emesis and hyperproteinemia remeined, despite medical therapy for the gastro-
Downloaded From: http://archpedi.jamanetwork.com/ by a Karolinska Institutet University Library User on 05/31/2015
esophageal reflux.
Just prior to operation, the fecal excretion of ''Cr-labeled albumin was mildly elevated at 2.8%. Inflammation of the full thickness of the esophagus and a localized reactive mediastinitis were noted during surgical repair of the hiatal hernia and gastroesophageal reflux. Intestinal blood loss stopped following operation, and the serum protein levels returned to normal in one week. Emesis and other symptoms of the rumination syndrome also ceased. Over the next nine months, the finger clubbing improved and the ratio of the depth of the distal phalangeal depth to the interphalangeal depth decreased from 1.020 preoperatively to 0.982.
COMMENT
Gastroesophageal reflux with hiatal hernia has been associated with un¬ usual presentations, including the rumination syndrome and Sandifer syndrome (reflux esophagitis, iron deficiency anemia, and head cock¬ ing)."7 Each of these unusual syn¬ dromes associated with gastroesopha¬ geal reflux was demonstrated in at least one of our three patients. In addition, all three had protein-losing enteropathy and finger clubbing. The gastroesophageal reflux and asso¬ ciated esophagitis did not respond to conservative therapy in our patients, and surgical correction of the reflux was necessary. The quick response fol¬
lowing surgical therapy helped to document the relationship between the gastroesophageal reflux with re¬ flux esophagitis and the associated symptoms of anemia, protein-losing enteropathy, finger clubbing, and rumination and Sandifer syndromes in our patients. It is possible that other patients may respond to medical therapy, and as in other cases of gastroesophageal reflux, an intensive course of medical therapy is indicated prior to resorting to operative man¬ agement.
The magnitude of the protein loss indicated by the ''Cr-labeled albu¬ min studies is much greater than could be accounted for by loss of whole blood into the intestinal tract, indicat¬ ing that there was a selective loss of proteins from the intestinal tract. The site of the protein loss was not detected by the methods used. The as
possibility that
the intestinal protein due to an obstruction of lymphatics in the thorax or to an intestinal mucosal lesion cannot be ruled out. However, the normal intes¬ tinal biopsy specimens, the lack of a specific roentgenographic abnormali¬ ty in the stomach or intestines, and the rapid increase in serum protein level after surgical correction of the gastroesophageal reflux in all three cases strongly suggest that the reflux esophagitis and protein-losing enter¬ opathy were related. Waring has proposed a simple, objective way of quantitating club¬ bing by measuring the ratio of the distal phalangeal-interphalangeal depth.' The determination of these ratios provided objective evidence that clubbing was present in all three. The child in case 2 was not available for measurement of the phalangeal ratios after operation. However, dur¬ ing the following year, his clubbing resolved. Finger clubbing has been associated with intrathoracic diseases, including cyanotic heart disease, chronic lung disease, and tumors of the lung and esophagus." Extrathoracic diseases associated with club¬ bing include tumors in the nasopha¬ rynx and stomach, cirrhosis of the liver, and chronic inflammatory bowel disease. On rare occasions, clubbing has been associated with achalasia of the esophagus, but these patients frequently have chronic aspiration pneumonitis.11 None of our patients had evidence of these other diseases associated with clubbing. Botha mentions two patients with hiatal hernia and finger clubbing in whom the clubbing resolved after operation.' Whether the presence of an inflamed, edematous esophagus with adjacent reactive mediastinitis was etiologically important remains conjectural, but the regression of club¬ bing after operation in our three cases and in Botha's additional two patients with hiatal hernia and gastroesopha¬ geal reflux suggests that the esopha¬ gitis may be an important factor. The occurrence of Sandifer syn¬ drome in case 1 is of interest. The head cocking trait distinguishes this
leak
was
from other cases of hiatal hernia associated with reflux esoph¬ agitis and iron deficiency anemia.'1 Possibly the head cocking trait repre¬ sents one of several unusual manifes¬ tations of severe gastroesophageal reflux. Others include rumination, finger clubbing, and protein-losing enteropathy. The cause of head cock¬ ing is unknown, but our cine-esophagram studies in case 1 indicate that head cocking occurred right after massive reflux up to the superior esophageal sphincter. Almost imme¬ diately after surgical correction of the reflux, the head cocking trait stopped. Interestingly, a previously described patient with hiatal hernia and rumi¬ nation manifested tongue-thrusting motions following reflux to the super¬ ior esophageal sphincter, which was documented by cine-esophagram.T
syndrome
Esophagitis secondary gastroesophageal reflux
to
severe
should be included in the differential of finger clubbing and protein-losing enteropa¬ thy. On the basis of our experience, we propose that Sandifer syndrome, ru¬
mination, protein-losing enteropathy,
finger clubbing be considered as an expanded syndrome of unusual presentations of severe gas¬ troesophageal reflux in children. and
part of
References 1. Rohatgi M, Shandling B, Stephens CA: Hiatal hernia in infants and children: Results of surgical treatment. Surgery 69:456-462, 1971. 2. Carre IJ: The natural history of the partial thoracic stomach (hiatal hernia) in children. Arch Dis Child 34:344-353, 1959. 3. Botha GS: Partial thoracic stomach in infancy, in The Gastroesophageal Junction. Boston, Little Brown & Co, 1962, pp 320-322. 4. Waring WW, Wilkinson RW, Wiebe RA, et al: Quantitation of digital clubbing in children. Am Rev Respir Dis 104:166-174, 1971. 5. Waldmann AT: Protein-losing enteropathy and kinetic studies of plasma protein metabolism. Semin Nucl Med 2:251-264, 1972. 6. Kinsbourne M, Oxon DM: Hiatus hernia with contorsions of the neck. Lancet 1:1058-1061, 1964. 7. Herbst J, Friedland GW, Zboralske FF: Hiatal hernia and "rumination" in infants and children. Pediatr 78:261-265, 1971. 8. Maurice-Williams RS, Wilson RJ: Hypertrophic osteoarthropathy associated with carcinoma of the oesophagus. Postgrad Med J 45:743\x=req-\ 744, 1969. 9. Peyman MA: Achalasia of cardia, carcinoma of oesophagus and hypertrophic pulmonary osteoarthropathy. Br Med J 1:23-25, 1959. J
Downloaded From: http://archpedi.jamanetwork.com/ by a Karolinska Institutet University Library User on 05/31/2015
Herbst, MD; Dale G. Johnson, MD; Miguel
\s=b\ Finger clubbing, protein-losing opathy, and iron deficiency were
mented in three children with
enterdocusevere
gastroesophageal reflux. One patient had Sandifer syndrome and the other two had the rumination syndrome. In each case, surgical repair of the gastroesophageal reflux resulted in immediate clearing of signs of the Sandifer syndrome, gastroesophageal reflux, and anemia and the return of serum protein levels to normal. There was definite regression of the finger clubbing during the ensuing year. It is suggested that finger clubbing, proteinlosing enteropathy, Sandifer syndrome, and rumination be viewed as parts of an extended syndrome of unusual presentations of gastroesophageal reflux. (Am J Dis Child 130:1256-1258, 1976) reflux with or without hiatal hernia has been associated with a number of complica¬ tions, including failure to thrive,
Gastroesophageal
anemia, aspiration pneumonitis,
se¬
esophagitis, and stricture.' We describe three children with hiatal hernia, severe reflux esophagitis, iron vere
-
deficiency anemia, finger clubbing,
From the departments of pediatrics (Drs Herbst and Oliveros) and surgery (Dr Johnson), University of Utah Medical Center, Salt Lake
City. Reprint requests to Department of Pediatrics, University of Utah Medical Center, 50 N Medical Dr, Salt Lake City, UT 84112 (Dr Herbst).
A.
Oliveros,
MD
and protein-losing enteropathy. We believe these are the first case reports of such associated conditions. The only previous association of hiatal hernia and clubbing of the fingers in the literature is a brief reference to it in two children by Botha. Protein-losing enteropathy is a relatively common phenomenon associated with many disorders of the gastrointestinal tract, but carcinoma of the esophagus is the only disease primarily affecting the esophagus with which it has been related previously. '
REPORT OF CASES Three children with finger clubbing, protein-losing enteropathy, and severe gastroesophageal reflux were studied. The clubbing was quantitated by measuring the distal phalangeal-interphalangeal ratio as proposed by Waring et al.' Proteinlosing enteropathy was diagnosed by measurement of fecal excretion of radioac¬
tive chromium ("Cr) following intravenous injection of a bolus of "''Cr-labeled albu¬ min."' Case 1.—A 4V2-year-old boy was referred to us because of microcytic anemia unre¬ sponsive to ferrous sulfate therapy. He had a history of frequent "spitting" since birth. During the 18 months prior to referral, he vomited at least once every other day. The emesis was frequently blood-stained, and he exhibited a tendency to cock his head, often assuming this peculiar posture while recumbent (Fig 1). He complained of pain in the upper sternal area that sometimes awoke him at night, and he often obtained
by drinking large amounts of milk. a pale, irritable child, who frequently cocked his head to one side. His height and weight were at the 50th percen¬ tile for age. Clubbing of the fingers was evident and the ratio of the distal phalangeal-interphalangeal depth was 1.030 (nor¬ mal less than 0.985). The hemoglobin level was 6.7 gm/100 ml and the hematocrit reading was 25.5%. Serum iron level was 6/ig/100 ml, and serum iron-binding capac¬ ity 221ug/100 ml. Total serum protein level was 4.3 gm/100 ml, and albumin 2.3 gm/ 100 ml. After intravenous injection of ''Crrelief He
was
=
labeled
albumin,
five-day
stool
21'??
was
detected in
a
sample (normal less than 2%). A cine-esophagram showed a large hiatal hernia and gross gastroesophageal reflux up to the superior esophageal sphincter, and reflux seemed to occur just prior to the act of head cocking. X-ray films of the chest and small and large bowel
were
normal,
=
as was a
small bowel
biopsy specimen. The symptoms, anemia, and serum protein concentrations did not improve following oral ferrous sulfate therapy and institution of intensive medi¬ cal treatment of the gastroesophageal reflux.
Esophagoscopy at the time of operation showed severe reflux esophagitis without stricture. At operation, the entire thick¬ ness of the esophagus was inflamed and edematous, with considerable local mediastinal reaction. A combined Borema and Nisson repair of the hiatal hernia and gastroesophageal reflux was performed. Postoperatively, the head cocking disap¬ peared almost completely within three days. The serum protein levels returned to
Downloaded From: http://archpedi.jamanetwork.com/ by a Karolinska Institutet University Library User on 05/31/2015
Fig 1.—Head-cocking
trait
(case 1).
Fig 2.—Esophagram demonstrating
Fig
3.—Clubbed
massive reflux and
(case 2).
Features Quantitation of
Age,
hiatal hernia
finger (case 3).
Major Clinical
Case
large
yr 4'/2 5'/2 11
Hemoglobin, gm/100 ml 6.7 6.6 7.6
Albumin,
gm/100
Fecal* s'Cr 21.0 11.6 2.8
ml
2.3 2.4 3.2
Finger Clubbing!
Preoperatively
Postoperatively
Syndrome
1.030 1.102 1.020
0.966
Sandifer Rumination Rumination
"Percent of intravenously administered bolus ofs Cr-labeled albumin detected In 0.985. fRatlo of distal phalangeal depth to interphalangeal depth.
five-day
0.982 stool
sample.
=
normal within two weeks, and his anemia cleared in six weeks with oral ferrous sulfate therapy. Within nine months, the ratio of the distal interphalangeal depth to the interphalangeal depth had returned to normal (0.966), and clubbing was not noted
clinically (Table). Case 2.—A 5y2-year-old white boy was referred to us with a severe microcytic anemia and a hemoglobin level of 6.6 gm/ 100 ml. He had vomited frequently since birth and had been taking ferrous sulfate since 9 months of age for chronic iron deficiency anemia. Two years previously, a hiatal hernia with free gastroesophageal reflux had been diagnosed (Fig 2), and positional therapy was initiated. He con¬ tinued to vomit at least three times a week; however, no history of head cocking was obtained. Physical examination showed a thin boy in the 50th growth percentile for
height and the 30th growth percentile for weight. He had definite clubbing of the fingers and toes, the ratio of the distal phalangeal depth to the interphalangeal depth being 1.102. Total serum protein level was only 5.2 gm/100 ml. Results of liver function tests were normal, and stool guaiac determina¬
tions were positive. X-ray studies revealed a normal chest, a large hiatal hernia, and free gastroesophageal reflux. Within one week of operative repair of the gastro¬ esophageal reflux, the intestinal blood loss stopped, the serum protein levels returned to normal, and emesis and other symptoms of the rumination syndrome ceased. Over the next nine months, the finger clubbing improved and the ratio of the depth of the distal phalangeal depth to the interphalan¬ geal depth decreased from 1.020 preoperatively to 0.982.
Case 3.—An 11-year-old mentally re¬ tarded boy was referred to us because of emesis, which had persisted since infancy. In recent months, it had increased in severity and he started to show the typical clinical signs of the rumination syndrome. Findings from physical examination were remarkable only in that he had finger clubbing (Fig 3). He had an iron deficiency anemia with a hemoglobin level of 7.6 gm/ 100 ml. Stool guaiac tests were positive. Serum protein level was 5.8 gm/100 ml and the albumin level was 3.2 gm/100 ml. He had radiological evidence of a large hiatal hernia and severe gastroesophageal reflux. Roentgenograms of the small and large bowel and a small bowel biopsy specimen were all normal. The patient's anemia improved after oral iron therapy, but the emesis and hyperproteinemia remeined, despite medical therapy for the gastro-
Downloaded From: http://archpedi.jamanetwork.com/ by a Karolinska Institutet University Library User on 05/31/2015
esophageal reflux.
Just prior to operation, the fecal excretion of ''Cr-labeled albumin was mildly elevated at 2.8%. Inflammation of the full thickness of the esophagus and a localized reactive mediastinitis were noted during surgical repair of the hiatal hernia and gastroesophageal reflux. Intestinal blood loss stopped following operation, and the serum protein levels returned to normal in one week. Emesis and other symptoms of the rumination syndrome also ceased. Over the next nine months, the finger clubbing improved and the ratio of the depth of the distal phalangeal depth to the interphalangeal depth decreased from 1.020 preoperatively to 0.982.
COMMENT
Gastroesophageal reflux with hiatal hernia has been associated with un¬ usual presentations, including the rumination syndrome and Sandifer syndrome (reflux esophagitis, iron deficiency anemia, and head cock¬ ing)."7 Each of these unusual syn¬ dromes associated with gastroesopha¬ geal reflux was demonstrated in at least one of our three patients. In addition, all three had protein-losing enteropathy and finger clubbing. The gastroesophageal reflux and asso¬ ciated esophagitis did not respond to conservative therapy in our patients, and surgical correction of the reflux was necessary. The quick response fol¬
lowing surgical therapy helped to document the relationship between the gastroesophageal reflux with re¬ flux esophagitis and the associated symptoms of anemia, protein-losing enteropathy, finger clubbing, and rumination and Sandifer syndromes in our patients. It is possible that other patients may respond to medical therapy, and as in other cases of gastroesophageal reflux, an intensive course of medical therapy is indicated prior to resorting to operative man¬ agement.
The magnitude of the protein loss indicated by the ''Cr-labeled albu¬ min studies is much greater than could be accounted for by loss of whole blood into the intestinal tract, indicat¬ ing that there was a selective loss of proteins from the intestinal tract. The site of the protein loss was not detected by the methods used. The as
possibility that
the intestinal protein due to an obstruction of lymphatics in the thorax or to an intestinal mucosal lesion cannot be ruled out. However, the normal intes¬ tinal biopsy specimens, the lack of a specific roentgenographic abnormali¬ ty in the stomach or intestines, and the rapid increase in serum protein level after surgical correction of the gastroesophageal reflux in all three cases strongly suggest that the reflux esophagitis and protein-losing enter¬ opathy were related. Waring has proposed a simple, objective way of quantitating club¬ bing by measuring the ratio of the distal phalangeal-interphalangeal depth.' The determination of these ratios provided objective evidence that clubbing was present in all three. The child in case 2 was not available for measurement of the phalangeal ratios after operation. However, dur¬ ing the following year, his clubbing resolved. Finger clubbing has been associated with intrathoracic diseases, including cyanotic heart disease, chronic lung disease, and tumors of the lung and esophagus." Extrathoracic diseases associated with club¬ bing include tumors in the nasopha¬ rynx and stomach, cirrhosis of the liver, and chronic inflammatory bowel disease. On rare occasions, clubbing has been associated with achalasia of the esophagus, but these patients frequently have chronic aspiration pneumonitis.11 None of our patients had evidence of these other diseases associated with clubbing. Botha mentions two patients with hiatal hernia and finger clubbing in whom the clubbing resolved after operation.' Whether the presence of an inflamed, edematous esophagus with adjacent reactive mediastinitis was etiologically important remains conjectural, but the regression of club¬ bing after operation in our three cases and in Botha's additional two patients with hiatal hernia and gastroesopha¬ geal reflux suggests that the esopha¬ gitis may be an important factor. The occurrence of Sandifer syn¬ drome in case 1 is of interest. The head cocking trait distinguishes this
leak
was
from other cases of hiatal hernia associated with reflux esoph¬ agitis and iron deficiency anemia.'1 Possibly the head cocking trait repre¬ sents one of several unusual manifes¬ tations of severe gastroesophageal reflux. Others include rumination, finger clubbing, and protein-losing enteropathy. The cause of head cock¬ ing is unknown, but our cine-esophagram studies in case 1 indicate that head cocking occurred right after massive reflux up to the superior esophageal sphincter. Almost imme¬ diately after surgical correction of the reflux, the head cocking trait stopped. Interestingly, a previously described patient with hiatal hernia and rumi¬ nation manifested tongue-thrusting motions following reflux to the super¬ ior esophageal sphincter, which was documented by cine-esophagram.T
syndrome
Esophagitis secondary gastroesophageal reflux
to
severe
should be included in the differential of finger clubbing and protein-losing enteropa¬ thy. On the basis of our experience, we propose that Sandifer syndrome, ru¬
mination, protein-losing enteropathy,
finger clubbing be considered as an expanded syndrome of unusual presentations of severe gas¬ troesophageal reflux in children. and
part of
References 1. Rohatgi M, Shandling B, Stephens CA: Hiatal hernia in infants and children: Results of surgical treatment. Surgery 69:456-462, 1971. 2. Carre IJ: The natural history of the partial thoracic stomach (hiatal hernia) in children. Arch Dis Child 34:344-353, 1959. 3. Botha GS: Partial thoracic stomach in infancy, in The Gastroesophageal Junction. Boston, Little Brown & Co, 1962, pp 320-322. 4. Waring WW, Wilkinson RW, Wiebe RA, et al: Quantitation of digital clubbing in children. Am Rev Respir Dis 104:166-174, 1971. 5. Waldmann AT: Protein-losing enteropathy and kinetic studies of plasma protein metabolism. Semin Nucl Med 2:251-264, 1972. 6. Kinsbourne M, Oxon DM: Hiatus hernia with contorsions of the neck. Lancet 1:1058-1061, 1964. 7. Herbst J, Friedland GW, Zboralske FF: Hiatal hernia and "rumination" in infants and children. Pediatr 78:261-265, 1971. 8. Maurice-Williams RS, Wilson RJ: Hypertrophic osteoarthropathy associated with carcinoma of the oesophagus. Postgrad Med J 45:743\x=req-\ 744, 1969. 9. Peyman MA: Achalasia of cardia, carcinoma of oesophagus and hypertrophic pulmonary osteoarthropathy. Br Med J 1:23-25, 1959. J
Downloaded From: http://archpedi.jamanetwork.com/ by a Karolinska Institutet University Library User on 05/31/2015
