Treatment of Congenital Microgastria and Dumping Syndrome

By Kathryn D. Anderson and Philip C. Guzzetta

Washington, DC

�9 Two children with congenital microgastria and associated anomalies requiring surgery as infants, developed severe dumping syndrome which necessi- tated a remedial operation. A Roux-en-Y jejunal pouch (Hunt-Lawrence) was formed at 22 months and 10 months respectively, Weight gain was imme- diate in both patients and has been sustained for 6 months and 8 years respectively. Symptoms of the dumping syndrome were relieved and the pouch delays filling of the jejunum.

INDEX WORDS: Congenital microgastria; dumping syndrome; jejunal pouch.

C ONGENITAL MICROGASTRIA was first noted in 1894 by Dide ~ and rede-

scribed as a "new syndrome" by Schulz and Newman in 1971.2 These latter authors observed skeletal abnormalities in association with the microgastria. This abnormality probably belongs in a constellation of anomalies which include megaesophagus, situs inversus, malrotation and asplenia. Thirteen examples have been recorded in the literature. Most infants present in the early days of life with vomiting, severe malnutrition and/or aspiration pneumonia. The present report offers two additional patients with congenital microgastria, who presented at birth and 10 months respectively. The surgical management of these infants is described and a follow-up of 8 years recorded for one of them.

CASE REPORTS

Case 1

D.H. was born in 1973 to a Gravida II, Para I healthy mother. The infant had dysmorphic features with right-sided anophthalmia and micrognathia. Excessive salivation in the first hours of life suggested esophageal atresia; however, a nasogastric tube passed into the stomach. Barium swallow showed a microstomach with a completely obstructed outlet (Fig. 1). These findings were confirmed at exploration. The small nonrotated stomach with an atretic outlet was anasto- mosed to the jejunum. The intestine was normally rotated. No spleen was identified.

Initially weight gain was slow but constant. At 13 months weight gain ceased and he developed bilious vomiting. The dumping syndrome appeared when the patient was begun on solid food. Surgical intervention was deferred because of a severe seizure disorder secondary to a porencephalic cyst.

At 21 months, a Hunt-Lawrence pouch was created using the afferent and efferent limbs of the gastrojejunostomy. Intestinal continuity was restored by an antiperistaltic anas- tomosis between the pouch and the distal jejunum (Fig. 2).

Fig. 1. Barium esophagram at birth, Case 1.

Postoperative weight gain has been highly satisfactory and vomiting and dumping have been eliminated. The child is now almost 10 years of age. Although below the third percentile, his weight gain is steady (Fig. 3) and he is able to cat large volumes of food without vomiting or dumping. Unfortunately his seizures are poorly controlled and he is grossly retarded as a result of his porencephalic cyst. By GI series the jejunal pouch functions as an effective reservoir.

Case 2

J.G. was first seen at CHNMC at 10 months of age because of vomiting and failure to thrive. There was no significant perinatal or infant history but she was below the fifth percentile for height and weight. UGI revealed a small stomach with massive GE reflux (Fig. 4).

At surgery she was noted to have microgastria with the majority of the stomach above the diaphragm. Malrotation and asplenia were also present. Extensive mobilization of the stomach from the posterior mediastinum was required to reposition the stomach beneath the diaphragm. A pyloro-

From George Washington University and the Children's Hospital National Medical Center, Washington, DC.

Presented before the 14th Annual Meeting of the Ameri- can Pediatric Surgical Association, Hilton Head Island, South Carolina, May 4-7, 1983.

Address reprint requests to Kathryn D. Anderson, MD, Professor of Surgery, George Washington University, Senior Attending Surgeon, Michigan Ave. N W Children's Hospital National Medical Center, Washington, DC.

�9 1983 by Grune & Stratton, Inc. 0022/3486/83/1806-0019 $01.00/0

Journal of Pediatric Surgery, Vol. 18, No. 6 (December), 1983 747

748 ANDERSON AND GUZZEI-rA

"h

B

Fig. 2. Hunt-Lawrence jejunal pouch. Note antiperistalt ic ana- stomosis at pouch outlet.

MICROGASTRIA AND DUMPING SYNDROME 749

Fig. 3. Case 1.

Operation

30 MONTHS 9.5 YEARS

Postoperative weight gain, birth to 91/2 years.

plasty was felt to be necessary because of probable injury to the vagus nerves during the dissection, A gastrostomy was placed and the stomach was sutured to the peritoneal side of the diaphragm. A Ladd procedure was performed to correct the malrotation.

Postoperatively the child had symptoms of dumping as well as persistent GE reflux. Dietary measures were recom- mended to combat the dumping and reflux. Despite a variety of feeding regimens, she failed to gain weight, At 15 months of age a Hunt-Lawrence pouch was constructed and sutured to the greater curve of her small stomach. She continued to have signs of early satiety which prevented weight gain. Night-time tube feedings were instituted to supplement her p.o. intake, She has no signs of GE reflux or dumping syndrome. She is developing normally and at 22 months of age her weight is above the 10% percentile.

DISCUSSION

Congenital microgastria is an extremely rare anomaly. Single case reports show that it is associated with many other visceral and skeletal anomalies including malrotation, situs inversus, transverse liver, absence of the gallbladder, and asplenia. 3'4 Associated skeletal abnormalities include hypoplasia of the radius and ulna and oligodactyly. 2 Micrognathia, anophathalmia, central nervous system anomalies, and pyloric atresia as manifested by our first case have not been described before. Most children present in infancy with gastroesophageal reflux, severe malnutrition, and growth retardation. Both of our patients manifested a severe dumping phe- nomenon which necessitated a surgical approach to increase "gastric" capacity and to delay tran- sit into the small bowel.

A number of different surgical procedures have been advocated to alleviate dumping and

Fig. 4. Preoperat ive UGI ser ies--Case 2 showing tho- racic microstomach, massive reflux and malrotat ion.

failure to gain weight following total or near- total gastrectomy. Most current procedures involve the creation of some kind of jejunal pouch as summarized by Herrington in 1970. 5 The Hunt-Lawrence pouch, modified by Poth, 6 has been extensively used by Scott. 7 A jejunal pouch was used successfully in a child by Neifeld, as reported in 1980, with a 1 year follow-up. 8 Our first patient had gastrojejunostomy at birth for pyloric atresia but required an additional opera- tion because of further vomiting and dumping. Creation of a jejunal pouch with an antiperistal- tic outlet 6 was delayed to age 22 months because of his severe seizure disorder. The second patient had her jejunal pouch with an isoperistaltic out- let at age 15 months after an unsuccessful attempt to control gastroesophageal reflux by gastropexy at 10 mouths of age.

Neither patient could tolerate feedings of more than 1 to 2 ounces of formula before the creation of a pouch. Postoperatively, vomiting and postprandial dumping stopped immediately and both children were able to tolerate bolus feeding. Patient J.G. (case 2) is still having some problems in oral feeding 6 months following surgery and needs supplemental feeding by NG tube. D.H. had similar difficulties for approxi- mately 1 year postoperatively; 8 years later he is able to eat large amounts of table food. Both patients began to gain weight steadily promptly after pouch construction. Consistent weight gai n ,

750 ANDERSON AND GUZZETTA

al though below the fifth percentile, has con- tinued for 8 years in the first patient (Fig. 3). Of interest is the fact that most of this retarded patient 's verbal development is associated with food and he appears to experience hunger at appropriate times. U G I series show a satisfac- tory capaci ty of the jejunal pouches with delayed emptying into the small bowel. Long- term fol- low-up demonstrates that the pouch grows with

the patient; delayed transit persists and reflux is diminished.

It is predictable that an increase in gastric capaci ty will be necessary in most patients with congenital microgastria. A jejunal pouch appears to offer satisfactory relief of symptoms and to provide adequate gastric capaci ty and function in growing subjects.

REFERENCES

1. Dide M: Sur en Estomac d'Adulte a Type Foetal Bull. Soc Anat Paris 69:669, 1894

2. Schulz RD, Nieman F: Kongenitale Mikrogastrie in Verbindung mit Skdetmissbidungen--ein neus Syndrom. Heir Paextiatr Acta 26:185-191, 1971

3. Blank E, Chisholm A J: Congenital microgastria, A case report with a 26 year follow-up. Pediatrics 51:1037-1041, 1973

4. Hochberger O, Swoboda W: Congenital Microgastria A follow-up observation over six years. Pediatr Radiol 2:207- 208, 1974

5. Herrington JL: Remedial operations for post-gastrec- tomy syndromes. Current Problems in Surgery, April, 1970

6. Poth E J: The dumping syndrome and its surgical treat- ment. Am Surg 23:1097-1102, 1957

7. Scott HW, Gobbel WG, Law DH: Clinical experience with a jejunal pouch (Hunt-Lawrence) as a substitute stom- ach after total gastrectomy. Surg Gynecol Obstet 1231- 1242, Dec, 1965

8. Neifeld JP, Berman WF, Lawrence W, et al: Manage- ment of congenital with a jejunal reservoir pouch. J Pextiatr Surg 15:882-885, 1980