Congenital microgastria

Congenital microgastria

Congenital Microgastria By Jeff C. Hoehner, Ken Kimura, and Robert T. Soper lo wa City, lo wa 0 Congenital microgastria is an extremely uncommon dys...

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Congenital

Microgastria

By Jeff C. Hoehner, Ken Kimura, and Robert T. Soper lo wa City, lo wa 0 Congenital microgastria is an extremely uncommon dysplasic condition of the newborn stomach. With only 26 well-documented accounts of this anomaly reported in the literature, its treatment and long-term outcome have not been well elucidated. Herein, the authors report on a newborn with multiple congenital anomalies who presented with severe reflux and aspiration. The workup showed congenital microgastria. The infant’s diagnostic and treatment course is described. Copyright o 1994 by W. B. Saunders Company INDEX WORDS:

Congenital

microgastria.

C

ONGENITAL microgastria is best characterized by a small, tubular stomach with a dilated esophagus and incompetence of the cardia.l-‘n Unfortunately, this developmental anomaly is usually accompanied by multiple additional life-threatening malformations involving the heart, lungs, gastrointestinal tract, and extremities.‘-lo Microgastria most often

leads to postprandial vomiting, gastroe$ophageal reflux, aspiration pneumonia, and malnutrition.‘-10 The clinical manifestations can vary significhtly, depending on the phase and degree to which stomach maturation is arrested. Various methods of gastrointestinal reconstruction (to provide relief from these complications and allow adequate entedal nutritional support) have been described. We describe a patient born with this entity and discuss her surgical treatment. CASE REPORT A baby girl was born at 37 weeks’ gestation to a healthy mother (gravida 1, para 1) who had had no prenatal complications. At birth, dysmorphic ears, frontal hypertrophy. and a loud systolic ejection murmur were evident. Sonographic examination of the chest and abdomen showed a persistent atriovedtricular canal, a midline liver, asplenia. and an annular pancreas. Initial attempts at oral feedings were not successful. A nasogastric /tube was passed for feeding purposes; however, attempts to gavade feed were met with emesis, choking, and life-threatening cyanosis. The patient was intubated and maintained by positive pressure ventilation. Serial chest x-ray examinations did not show as iration pneumonia. An upper gastrointestinal (UGI) contrast stu% y (Figs 1 and 2) showed microgastria. incompetence of the cardi and indications of annular pancreas. The intestinal anatomy was1otherwise unremarkable. The patient was sustained on central venous nutrition. On the 25th day of life, a laparotomy confirmed t$e above findings (Fig 3). A Roux-en-Y feeding jejunostomy and, an end-cervical esophagostomy (spit fistula) were constructed (Fig 4). The patient recovered uneventfully, and enteral feeding through the jejunostomy was begun on postoperative day (POD) 3. Gral sham feeding was initiated on POD 5. Jejunal feedings were advanced to full nutritional requirements by POD 6, and the patient was discharged at 35 days of age. Subsequently, she was admitted because of respiratory syncytial virus and required ventilatdry support. Her growth has been maintained in the 90th percentile. DISCUSSION

This patient typifies individuals born with congenital microgastria. A constellation of congenital anomalies in association with a small gastric reservoir, gastroesophageal reflux, and aspirationiare the usual presenting characteristics. With this condition, the stomach assumes a small, saccular, or r/ubular shape in a midsagittal position, with minimal reservoir

Fig 1. UGI contrast study shows a small, tubular, stomach, consistent with congenital microgastria. JournalofPediafric

Surgery, Vol29, No 12 (December), 1994: pp 1591-1593

From the Deparrment qf SuTety, Universi~ of Iowa Hospitals and Clinics, Iowa City, IA. Address reprint requests to Ken Kimura, MDi Department of Srqety, John CoNoton Pavilion. Universi~ of i&a Hospitals and Clinics, Iowa City, IA 52242. Copyright CI1994 by W B. Saunders Company 0022-.~4~Xf94l2912-0031$0.~,0~J0

1591

HOEHNER, KIMURA, AND SOPER

Fig 2. Contrast roentgenogram and duodenum.

of the distal esophagus, stomach,

capacity. The greater and lesser curvatures are poorly defined, but there is normal muscular and glandular development.’ It is postulated that congenital microgastria results from arrested gastric development during the first trimester of prenatal life.lJl

Fig 3. Appeerance at laparotomy of a patient with congenital microgastria. Note the midline liver (black arrow), enlarged duodenum (white arrow), and small atrophic stomach.

Fig 4. End esophagostomy constructed for this patient.

and Roux-en-Y feeding jejunostomy, as

Congenital microgastria was first reported in 1894 by Dide, who noted a small, atypically oriented stomach in an epileptic woman during autopsy.‘* In 1971, Schultz and Nieman described the condition in an infant with multiple organ atresias.13 Gerbeaux et al described the first surgical treatment for this entity by intestinal loop interposition to enlarge the “stomach.“14 Since then, treatment has consisted of conservative nonsurgical observation for patients who are less severely afflicted. Various forms of surgical treatment have been used to provide improved enteral nutrition in individuals with more severe dysplasia 4-5,9-10 Treatment of congenital microgastria must be individualized. In patients with lesser degrees of gastric aplasia, conservative nonsurgical treatment with small frequent feedings has had good results.4,6-7In patients with more severe dysgenesis, a conservative approach typically is unsuccessful. Bypass or feeding jejunostomy has been reported for cases with long-term survival.8~9Creation of a Hunt-Lawrence pouch has been used to enlarge gastric reservoir capacity.5,9-10

CONGENITAL

1593

MICROGASTRIA

Construction of a gastric pouch not only lessens the frequency of feedings and improves nutritional intake, but also facilitates drainage and prevents alkaline refiux esophagitis. The largest series to date was reported by Velasco et al.9 They described four patients with congenital microgastria. Three of them were treated with Hunt-Lawrence pouch reconstruction, with good results; the other died of associated anomalies before surgical treatment. Including the present case, all five patients treated with HuntLawrence reconstruction are alive and well; however,

their growth and weight gain have been moderately to severely retarded. The present patient, who had congenital microgastria, gastroesophageal reflux, and unacceptable oral intake, initially was treated with cervical esophagostomy and Roux-en-Y feeding jejunostomy. Such treatment permits adequate weight gain and growth and allows for training in oral feeding. Esophagostomy takedown and gastric augmentation is planned at 1 to 2 years of age, as the child matures and her cardiopulmonary status stabilizes.

REFERENCES 1. Simstein N: Congenital gastric anomalies. Am Surg 52:264267. 1986 2. Shackelford G, McAlister W, Brodeur A, et al: Congenital microgastria. AJR 118:72-76, 1973 3. Kessler H, Smulewicz J: Microgastria associated with agenesis of the spleen. Pediatr Radio1 107~393-396,1973 4. Aintablian A, Slim M, Antoun B: Congenital microgastria: Case report and review of the literature. Pediatr Surg Int 2:307310, 1987 5. Neifield JP, Berman WF, Lawrence W, et al: Management of congenital microgastria with a jejunal reservoir pouch. J Pediatr Surg 15882-885, 1980 6. Gorman B, Shaw DG: Congenital microgastria. Br J Radio1 571260-262, 1984 7. Hochberger 0, Swoboda W: Congenital microgastria: A follow-up observation over six years. Pediatr Radio1 2207-208, 1974

8. Bland E, Chisolm A: Congenital microgastria, a case report with a 26-year follow-up. Pediatrics 51:1037-1041. 1973 9. Velasco A, Holcomb G, Templeton G, et al: Management of congenital microgastria. J Pediatr Surg 25:192-197, 1990 10. Anderson K, Guzzetta P: Treatment of congenital microgastria and dumping syndrome. J Pediatr Surg 18:747-750, 1983 11. Langman J: Medical Embryology (ed 2). Baltimore, MD, Williams &Wilkins, 1981, pp 212-222 12. Dide M: Sur un estomac d’adulte de type foetal. Bull Sot Anat (Paris) 69:669-670, 1894 13. Schultz RD, Nieman F: Kongenitale mikrogastrie in verbindung mit skeletmibbildungen-Ein neues syndrom. Helv Pediatr Acta 26:185-191.1971 14. Gerbeaux J, Couvreur J, Vialas M, et al: Absence congenitale d’estomac. Ann Pediatr 18:349-356, 1971