Congenital microgastria is a rare condition first reported in the 1800s (1,2). To date, fewer than 40 cases have been reported. The malformation has been frequently associated with other congenital anomalies including asplenia, malrotation of the intestines, situs inversus, megaesophagus (3-5), and upper limb anomalies such as radial, ulnar, and thenar hypoplasia (microgastria-limb reduction association) (2,6). Reported therapy for microgastria has ranged from conservative dietary manipulation in patients minimally affected, to surgical augmentation of the gastric cavity. The latter approach has been most commonly advocated in the more recent literature. Surgery is the usual approach to caring for these patients. We report a patient with microgastria-limb reduction association successfully treated with minimal surgical intervention and aggressive nutritional support, despite initial intolerance of oral feedings.
The patient, a boy, was born after full-term gestation complicated by third-trimester maternal polyhydramnios. A prenatal ultrasound performed at the onset of polyhydramnios revealed a small fetal gastric bubble, suggesting congenital microgastria. Birth weight was 4.28 kg (>95th percentile). No gastric bubble was seen on an abdominal roentgenogram obtained after birth, and an upper gastrointestinal barium study confirmed the prenatal diagnoses of microgastria, showed free reflux of the barium column into the esophagus, and showed normal intestinal rotation. Other anomalies detected during the postnatal period included right ulnar deviation, left thenar hypoplasia, and web malformation of the left thumb. Additionally, he had scoliosis, left deviating torticollis, and an undescended left atretic testicle.
Oral feeding was initiated but resulted in low volume of intake and frequent emesis. Consequently, gastroenterology and surgery were consulted on day 3 of life for management of the microgastria and significant emesis with feedings. Therapy included 0.3 mg/kg cisapride four times per day and 2 mg/kg ranitidine two times per day with ad libitum oral feedings of cow's milk-based formula. At 3 weeks of age the patient's weight was 4.15 kg (0.13 kg below birth weight), and he was consuming only 13 oz (94 ml/kg, 63 kcal/kg) per day of formula, although he was fed every 1 to 2 hours through the night. He had occasional episodes of vomiting. The formula concentration was increased to 25 kcal/oz and he was reevaluated at 5 weeks of age. Although his weight had increased to 4.4 kg, his oral intake had not increased. His parents were exhausted, and he was showing early signs of dehydration. Consequently, nocturnal nosojejunal (NJ) feedings were initiated, in which he received 10 oz over 12 hours, with oral feedings continuing during the day.
The patient's oral intake of formula continued to increase, he began consuming solid food, and his nighttime NJ feedings were decreased to 4 oz at 6 months of age, maintaining 100 kcal/kg per day of intake. At age 7 months, however, he was admitted with an intercurrent diarrheal and respiratory viral illness. During the illness, his weight decreased from the 15th to less than the 5th percentile (7.1 kg down to 6.8 kg), and his oral intake decreased significantly. His oral intake did not recover after approximately 1 month. Anticipating a prolonged requirement for supplemental nocturnal feedings, a jejunal skin-level feeding device (JSLD) was placed surgically (18 French, 1.7 cm, Bard gastrostomy button).
During subsequent months, his oral intake continued to increase, and he made the transition to whole cow's milk and table food. The concentration of the nocturnal jejunal feedings was decreased to 20 kcal/oz, and the volume decreased as his oral intake increased. By 22 months of age his antireflux medications were discontinued, and he was consuming a normal toddler's diet without supplementation. His weight and height were between the 15th and 25th percentiles. By 27 months of age, his height had increased to 89.5 cm (50%), and his weight had increased to 11.7 kg (15%-20%) with a normal diet. His JSLD was surgically removed approximately 3 months later.
In the fourth embryonic week of development, the primitive stomach appears as a fusiform dilatation of the foregut. During the following weeks it rotates 90° around its longitudinal axis with the original posterior wall of the stomach growing faster than the anterior wall, resulting in the formation of the greater and lesser curvatures, respectively (7). Early arrest of this normal developmental process results in microgastria, seen as a small tubular or saccular midline stomach. The theory of arrested development is supported by the finding of failure of macroscopic development of the fundus, corpus, and antrum (8,9). Some have reported differentiation of these morphologic regions, however, with time (10). As might be expected, this developmental arrest appears to be limited to anatomic development, with the mucosal cellular differentiation appearing normal, but with reduced total cell mass (5,8,9,11).
Unlike our patient, most patients are outside of the neonatal period at the time of diagnosis, frequently have a dilated esophagus with an ill-defined gastroesophageal junction, and are therefore prone to gastroesophageal reflux (GER). Consequently, patients with microgastria usually have poor growth, inadequate food consumption, or a complication of the small gastric cavity and GER, including frequent vomiting or pneumonia. This and other cases emphasize the need for a upper gastrointestinal barium study to evaluate patients with excessive vomiting in the infant age group. Because microgastria was diagnosed in utero in our patient, therapy could be initiated before nutritional and GER-related complications developed.
Reported management of patients with microgastria has ranged from conservative dietary manipulation (small, frequent feedings) to reconstructive gastric surgery. Although few, there are reports that conservative dietary manipulation in patients with significant microgastria has resulted in ultimate tolerance of small, frequent feedings, but patients continue to have long-term growth retardation (8,10). Most reports have shown that over a few years the stomach grows and is better able to accommodate ingested food, and the previously dilated esophagus seems to resume a normal size (1,10). In our patient, malnutrition was prevented by early supplemental jejunal feedings.
Several surgical procedures have been undertaken to permit increased nutritional intake and/or increased gastric capacity. Formation of a gastrostomy or gastroenterostomy has resulted in limited success because of continued GER (9). In 1980 Neifeld et al. (12) reported the construction of a double-lumen jejunal (Hunt-Lawrence) pouch in a patient with congenital microgastria. Originally used in the restoration of gastrointestinal continuity in patients who had undergone total gastrectomy (9), the procedure involves creation of a pouch on the stomach, increasing its cavity size. Since its initial use for microgastria, the Hunt-Lawrence pouch has become the most popular reconstructive surgery for this condition (9,11-15). Unfortunately, patients who undergo this procedure may continue to have poor nutritional outcome (13-15).
Our patient was initially approached conservatively with small frequent feedings, but was unable to maintain adequate growth and hydration, similar to previously reported patients. Because supplemental gastric feedings could have resulted in increased GER and risk of aspiration, a nasojejunal tube was placed. The patient had good weight gain and growth with supplemental nocturnal feedings, in addition to ad libitum daytime oral feedings. However, during this early period, he was still unable to increase his oral intake sufficiently to maintain his weight gain and growth without supplemental tube feedings, and consequently, a JSLD was surgically placed. Approximately 1 year after the JSLD was placed, the patient was maintaining good weight gain and growth, and his oral intake had increased enough that supplemental jejunal feedings were discontinued. Placement of a jejunal feeding device in patients with microgastria may be a viable alternative to gastric augmentation surgery, especially early in life when stomach growth is not complete. Furthermore, jejunal feedings may allow sufficient time to establish adequate oral intake while stomach growth ensues and yet still permit gastric augmentation surgery in the future should it be required.
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