Congenital heart disease in adolescents with gluteal muscle contracture.

Gluteal muscle contracture (GMC), presented with hip abduction and external rotation when crouching, is common in several ethnicities, particularly in Chinese. It remains unclear that the reasons why these children are weak and have no choice to accept repeated intramuscular injection. Here, we found some unique cases which may be useful to explain this question. We describe a series of special GMC patients, who are accompanied with congenital heart disease (CHD). These cases were first observed in preoperative examinations of a patient with atrial septal defect (ASD), which was proved by chest X-ray and cardiac ultrasound. From then on, we gradually identified additional 3 GMC patients with CHD. The original patient with ASD was sent to cardiosurgery department to repair atrial septal first and received arthroscopic surgery later. While the other 3 were cured postoperative of ventricular septal defect (VSD), tetralogy of fallot (TOF), patent ductus arteriosus (PDA), respectively, and had surgery directly. The study gives us 3 proposals: (1) as to CHD children, it is essential to decrease the use of intramuscular injection, (2) paying more attention to cardiac examination especially cardiac ultrasound in perioperative period, and (3) taking 3D-CT to reconstruct gluteal muscles for observing contracture bands clearly in preoperation. However, more larger series of patients are called for to confirm these findings.

INTRODUCTION

GMC, also named “injection contracture,” is a clinical syndrome characterized by gait abnormality and limb dysfunction including restriction of adduction and internal rotation of the hip joint.[1] It was first found by Valderrama at a scientific meeting of the British Orthopedic Association in London in 1969.[2] Its pathologic change typically presents with fibrosis and contracture of the gluteus and its fascia.[3] The knees cannot be brought together and are separated in a frog-leg position.[4] As younger patients with GMC continue to develop and grow, pathologic changes increase in prevalence and severity including leg length discrepancy, pelvic oblique, compensatory scoliosis, and, in severe cases, bilateral dislocation of the hip joints.[5] Now, arthroscopic release, a minimally invasive surgery which was reported by Zhang et al for the first time,[6] has became the gold standard of treatment in GMC patients.[4,7]

It is reported that GMC is related to benzyl alcohol. Compared with developed countries, GMC is more widely reported in China. So far, numerous studies have suggested that GMC usually was associated with repeated intramuscular injection into the gluteal region during childhood.[8-12] However, it remains unclear that, the reasons why these children are weak and have no choice to accept repeated intramuscular injection.

The paper reveals that CHD children are prone to catching cold, repeated respiratory tract infection, or pneumonia and have to take frequently intramuscular injection which use benzyl alcohol as a dissolvent for penicillin in some regions of China in the 1970s and 1980s, leading to degeneration, necrosis and fibrosis of the gluteal muscles and fascia, as well as serious limitation of hip movements.

PATIENTS AND METHODS

We identified 4 GMC adolescents with CHD from January 2013 to March 2014. The duration of symptoms ranged from 6 to 10 years. All had been unable to improve with functional exercise. There were 2 males and 2 females with age ranging from 14 to 17 years (Table 1). All patients provided informed consent for the operation and followed our rehabilitation program. This study was approved by the Institutional Review Board of our institution. All patients had a history of injections around the gluteal region. Although 3 of 4 patients (patients 1, 3, and 4) had limitations with activities of daily living, they believed their activity levels were lower than those of healthy people. Three of 4 patients (patients 1, 3, and 4) had an abnormal gait with out-toe walking, and could not crouch with both knees close to each other or sit down with their legs crossed. All patients had a snapping sound during rotation of the hip. Patient 1 was diagnosed as CHD for the first time, while the other 3 had been cured yet. Patient 4 had received open surgery for GMC but dissatisfied result. Two representative patients (Patients 1 and 4) were selected for further discussion of their clinical and radiographic findings (Table 2).

TABLE 1

Clinical Characteristics of the Patients

TABLE 2

Radiographic Findings of the Patients

Each author certifies the study had approved, by Ethics committee of Peking University Shenzhen Hospital, for the reporting of these cases and that all investigations were conducted in conformity with ethical principles of research.

CASE REPORTS

Case 1

Patient 1 was a 16-year-old girl who had bilateral diseases with gluteal muscle atrophies, abnormal gait with out-toe walking, dimpling of skin around the gluteal regions, snapping hips, adduction and internal rotation dysfunction of hips, Ober's signs. The most thing affected her was adduction and internal rotation dysfunction of hips, which made her not to do competitive sports (actually, it was mainly influenced by CHD which was diagnosed later.) like other healthy girls. 3D reconstruction CT showed that her gluteus maximus and gluteus medius were obviously dysplastic and part of them turned to contractile bands. We heard a systolic ejection murmur at the second left intercostal space in preoperative examinations. Furthermore, Chest X-ray revealed pulmonary vascularity, cardiac enlargement and aortic knob shrinking. Finally, cardiac ultrasound proved the diagnosis of ASD. Because of this, we sent her to cardiosurgery department for atrial septal defect closure and partial anomalous pulmonary venous drainage correction on beating heart. The arthroscopic release and rehabilitation program for GMC were operated 110 days later, when she was completely rehabilitated from cardiosurgery. Three months later, the patient gained a satisfactory result without complications, snappings, and dysfunctions of hip (Figure 1).

FIGURE 1

(A) A 3D reconstruction CT shows the gluteal muscles of Patient 1, a 16-year-old woman who had GMC with severe gluteus maximus and gluteus medius atrophy. (B) and (C) show the contracture bands (white arrow) in left and right side, respectively. (D) Cardiac ultrasound demonstrates ASD. (E) Chest X-ray reveals pulmonary ascularity, cardiac enlargement and aortic knob shrinking.

Case 2

Patient 4 was a 17-year-old boy who had bilateral diseases with gluteal muscle atrophies, abnormal gait with out-toe walking, dimpling of skin around the gluteal regions, snapping hips, adduction and internal rotation dysfunction of hips, Ober's signs. The most things affected him was adduction and internal rotation dysfunction of hips, as well as snapping hips. Due to a failed open surgery before, he decided to choose the minimally invasive surgery. When he was very young, he was taken ligation of patent ductus arteriosis (the detailed data of that surgery were unavailable). Physical examinations and chest X-ray were normal. 3D reconstruction CT showed that his gluteus maximus and gluteus medius were obviously dysplastic and part of them turned to contractile bands. Then, we implemented the arthroscopic release and rehabilitation program of GMC. Three months later, the patient gained a satisfactory result without complications, snappings, and dysfunctions of hip (Figure 2).

FIGURE 2

(A) A 3D reconstruction CT shows the gluteal muscles of Patient 4, a 17-year-old man who had GMC with mild gluteus maximus and gluteus medius atrophy. (B) and (C) show the contracture bands (white arrow) in left and right side, respectively. (D) Chest X-ray reveals normal.

DISCUSSION

We present a series of 4 GMC patients with CHD, including ASD, VSD, TOF, and PDA respectively. Accordingly, we suggest that some GMCs are related to CHDs, or rather are derived from CHDs. To be specific, children suffered from CHDs are easy to acquire respiratory infections (nearly one-quarter to one-third[13]), which may be result in frequent use of benzyl alcohol as a dissolvent for penicillin for intramuscular injections in some regions of China in 1970s–1980s, as several researchers have suggested that benzyl alcohol is the main cause of GMC.[11-12]

This series is limited by its small size relating to the decreasing of GMC for abandoning benzyl alcohol as a dissolvent. Patient 1 was admitted to hospital for releasing GMC, however, ASD was found in preoperative examinations. This warns that surgeons should attach more importance to the cardiac examination, especially identifying the real cause of “cannot do competitive sports.” When CHD is repaired and physical condition is fully recovered, it is time to release GMC. At last, the patient acquired an excellent outcome without complications. Patient 4 was hospitalized in order to cure GMC through a minimally invasive surgery for a noneffective open surgery 4 years ago. With the normal results of physical examinations and chest X-ray, we did arthroscopic release directly, like other common GMC patients without CHD. Eventually, he also had an excellent outcome without complications.

CONCLUSIONS

GMC with CHD is uncommon. Although there are no exact cause–result relationship between the 2 diseases, we propose 3 recommendations: (1) as to CHD children, in addition to treating the primary disease as early as possible, it is essential to decreasing the use of intramuscular injection, and (2) paying more attention to cardiac examination especially cardiac ultrasound in perioperative period, and (3) taking 3D-CT to reconstruct gluteal muscles for observing contracture bands clearly in preoperation.