Mici-ogastria as an Isolated Anomaly By Carmen T. Ramos, R. Lawrence Moss, and Catherine A. Musemeche

Albuquerque, New Mexico

l The authors report the case of an B-year-old boy who had isolated microgastria. The patient had required lifelong jeju- nal tube feeding because of inadequate gastric volume and intractable gastroesophageal reflux. Gastric augmentation was performed with a Roux-en-Y jejunal reservoir (Hunt- Lawrence pouch), with good results. Copyright o 1996 by W. B. Saunders Company

INDEX WORDS: Isolated microgastria, gastroesophageal re- flux, Hunt-Lawrence pouch.

M ICROGASTRIA has been described as a rare congenital anomaly of normal foregut develop-

ment. The microstomach has the following adverse effects: gastroesophageal reflux, vomiting, aspiration, malnutrition, failure to thrive, and in severe cases, death. It is almost always associated with other anomalies such as VACTERL syndrome (vertebral anomalies, imperforate anus, cardiac anomalies, tra- cheoesophageal fistula, esophageal atresia, renal and limb anomalies), malrotation, cardiorespiratory fail- ure, and asp1enia.i.* All but one female patient of the 38 cases of microgastria described in the literature had associated congenital anomalies.9 This is the first male patient, to our knowledge, with microgastria but no other congenital anomalies.

CASE REPORT

An g-year-old boy presented with inability to feed orally and with failure to thrive. At 1 month of age, he had been unable to tolerate oral feeding because of severe reflux. Medical treatment failed, and he underwent an attempt at fundoj$ication and gastrostomy tube placement at 6 months of age. Microgastria was identified at the time of operation. He had several subsequent admissions to the hospital because of aspiration pneumonia. His gastroesophageal reflux was so severe that it resulted in strictures of the posterior nasopharynx. A jejunal feeding tube was placed. Repeated upper gastrointestinal series showed failure of the stomach to enlarge, associated with severe reflux (Fig 1).

At the time of presentation, the patient was not able to tolerate any oral feeding, and jejunostomy tube feeding had been used for 7 years. Upper endoscopy showed a microstomach, with a scarred, fibrotic mucosa and an obstructed pyloric channel. We performed a subtotal gastrectomy and gastric augmentation using a Roux-en-Y jejunal pouch. The pouch was sewn to the stomach in end-to-side fashion. Currently, the patient is able to tolerate oral feedings and has no evidence of reflux.

DISCUSSION

The outcome for children with microgastria is still uncertain because of associated major congenital anomalies that preclude oral intake and normal quality of life (Table 1).

Normally, the stomach undergoes a 90” clockwise rotation in the fifth week of embryological develop- ment. This occurs concomitantly with the migration of the pancreatic buds and the differentiation of the dorsal mesogastrium into the splenic anlage. Micro- gastria will occur as a result of an arrest in the early development of the foregut and an abnormal migra- tion at the primitive streak stage. Deficiencies in the formation of the dorsal mesogastrium will lead to splenic anomalies.2,3~5,10J1

Microgastria also has been associated with limb reduction defects. Cuniff et al suggested that the etiology for these various defects could be an isolated defect in limb development or the presence of a multiple malformation syndrome.6 All the limb de- fects have been reported for the upper limbs, such as hypoplasia and agenesis. The affected structures are of mesodermal origin and are forming during the fifth and sixth embryonic weeks.4,6J2

Patients who survive with this condition suffer from malnutrition and failure to thrive. The creation of a food reservoir in patients with microgastria has proven to be effective in permitting these patients to grow normally and to achieve good quality of life. Success has been achieved with a gastric augmentation proce- dure, the Hunt-Lawrence pouch.5,7~8J3J4 Not all pa- tients with microgastria require augmentation. A few of them are able to tolerate enteral feeding with diet modification, ie, frequent and small-size meals.

Postprandial symptoms after total gastrectomy, such as dumping, epigastric pain, steatorrhea, fat malabsorption, and bacterial overgrowth, have been reported.5,8J0.14 We did not observe any of these symptoms in our patient. Vitamin supplementation, especially monthly vitamin B12 injections, is required because of the lack of intrinsic factor. Prophylactic penicillin is essential for patients with asplenia.

The prenatal presentation of microgastria is similar to that of esophageal atresia. Failure of visualization of the fetal stomach by the early part of the second trimester suggests the possibility of congenital micro-

From the Department of Surgery, The University of New Mexico Health Sciences Center, Albuquerque, NM.

Address reprint requests to Catherine A. Musemeche, MD, Division of Pediatn’c Surgery, Depatiment of Surgery The University of New Mexico Health Sciences Center, Albuquerque, NM 87131.

Copyright o 1996 by W.B. Saunders Company 0022-3468l9613110-0031$03.OOlO

JournaloffediafricSurgery, Vol31, No 10 (October), 1996: pp 1445-1447 1445

1446

Fig 1. Upper gastrointestinal series showed microgastria at the age of 6 years.

gastria or esophageal atresia.15 Other possible anoma- lies include diaphragmatic hernia, cleft palate, karyo- typic abnormalities, and central nervous system disorders that impair swallowing. If further prenatal workup results are consistent with microgastria, a thorough evaluation will be required when the infant is born.

When microgastria occurs as an isolated anomaly, we recommend early gastric augmentation because it is unlikely that the stomach will enlarge spontane- ously or grow. The patient’s age, the severity of symptoms, and the associated anomalies are key factors in determining the timing of gastric augmenta-

RAMOS, MOSS, AND MUSEMECHE

Table 1. Associated Anomalies

Author No. of

Year Patients Associated Anomalies

Robet+ 1842 1

Dide*O 1894 1 Peterma+ 1932 1

Caffeyz3 1956 1

Putschar et aI’9 1956 6

Schulz et aI24 1970 1

Kessler et al* 1973 1

2

1

1

1

2

1

1

1

1

4

3

1 1

3

1

1

1

Shakelford et a13 1973

Blank et al’

Hochberger et aI’* 1973 1974

Neifeld et a113 1980

Anderson et allo 1983

Mandell et aIt1 1983 Dorney et all6 1987

Gorman et al9 1984 Lueder et al4 1989

Velasco et al5 1990

1991

1991

1993

1993

1994

1994

1994

Ryan et al7

Reymunde et all7

Hasegawa et alI4

Cuniff et al6

Christianson et aIT2

Hill’5

Moulton et al8

Asplenia; forearm and wrist anomalies

Malrotation; epilepsy Asplenia; left renal agenesis

Malrotation Asplenia

Foreatm, wrist, and hand anomalies

Abnormal lung lobation;

ectopic right subclavian artery

Malrotation; asplenia; Hirsch- sprung’s duodenal atresia

Malrotation

Bilateral cryptorchidism; inguinal hernia

Malrotation

Asplenia; porencephalic cyst; malrotation

Splenogonadal fusion Complete agastria; cleft soft

palate None

Hypoplastic upper limb anomalies

Asplenia; malrotation; dia-

phragmatic hernia; tracheal stenosis; agenesis of right

lung Malrotation; laryngotracheo-

esophageal clefts; myelo- meningocele

Mute; deaf; global aphasia Malrotation; bilateral cystic

kidneys Renal anomalies; asplenia;

limb anomalies Acrofacial dysostosis syn-

drome (fetus) Malrotetion; hiatal hernia;

polysplenia

VACTERL association

tion. We agree with other investigators that the Hunt-Lawrence pouch is an excellent approach to the treatment of microgastria.

REFERENCES

1. Blank E, Chisolm AJ: Congenital microgastria, A Case 5. Velasco AL, Holcomb GW, Templeton JM, et al: Manage- Reuort with a 26-vear follow up. Pediatrics 51:1037-1041,1973 ment of congenital microgastria. J Pediatr Surg 25:192-197,199O

2. Kessler H, Smulewicz JJ: Microgastria associated with agen- esis of the spleen. Radiology 107:393-396,1973

6. Cuniff C, Williamson-Kruse L, and Haskins A: Congenital microgastria and limb reduction defects. Pediatrics 91:1192-1194, 1993

3. Shackelford CD, McAlister WH, Brodeur AE, et al: Congeni- tal microgastria. AJR 118:72-76,1973

7. Ryan DP, Muehrcke DD, Doody DP, et al: Laryngotracheo- esophageal cleft (type IV): Management and repair of lesions

4. Lueder GT, Fitz-James A, Dowton SB: Congenital microgas- beyond the carina. J Pediatr Surg 26:962-970, 1991

tria and hypoplastic upper limb anomalies. Am J Med Genet 8. Moulton SL, Bouvet M, Lynch FP: Congenital microgastria in 32~368-370.1989 a premature infant. J Pediatr Surg 29:1594-1595,1994

MICROGASTRIA

9. Gorman B, Shaw DG: Congenital microgastria. Br J Radio1 57:260-262, 1984

10. Anderson KD, Guzetta PC: Treatment of congenital micro- gastria and dumping syndrome. J Pediatr Surg 18:747-750, 1983

11. Mandell GA, Heyman S, Alavi A, et al: A case of microgas- tria in association with splenic gonadal fusion. Pediatr Radio1 13:95-98, 1983

12. Christianson AL, Kruger H, Dini L: Atypical acrofacial dysostosis syndrome. Am J Med Genet 51:32-34,1994

13. Neifeld JP, Berman WF, Lawrence W, et al: Management of congenital microgastria with a jejunal reservoir pouch. J Pediatr Surg 15:882-885,198O

14. Hasegawa S, Kohno S, Tamura K, et al: Congenital microgas- tria in an infant with the VACTERL association, J Pediatr Surg 28~782-784, 1993

15. Hill LM: Congenital microgastria: Absence of the fetal stomach and normal third trimester amniotic fluid volume. J Ultrasound Med 13:894-896,1994

16. Dorney SFA, Middleton AW, Kozlowski K, et al: Congenital agastria. J Pediatr Gastroenterol Nutr 6:307-310,1987

17. Reymunde A, Leon R: Congenital microgastria in an adult. Am J Gastroenterol86:1095-1096,199l

18. Hochberger 0, Swoboda W: Congenital microgastria, a follow up observation over six years. Pediatr Radio1 2:207-208,1974

19. Putschar WGF, Manion WC: Congential absence of the spleen and associated anomalies. Am J Clin Path01 26:429-470, 1956

20. Dide M: Sur un estomac d’adulte a type fetal. Bull Sot Anat 69:669-670, 1894

21. Robert HLF: Hemmungsbildung des magens, mange1 der milz und des netzes. Arch Anat Physiol Wissenschahiche Med 57-60,1842

22. Peterman MG: Congenital absence of spleen and left kidney. JAMA 99: 1252,1932

23. Caffey J: Pediatric X-Ray Diagnosis (ed 6). Chicago, IL, Year Book, 1973

24. Schulz RD, Niemann F: Kongenitale Mikrogastrie in verbind- ung mit skeletmibbildungen-ein neues syndrom. Helv Pediatr Acta 26:185,1971